Stanley Prusiner, MD

Title(s)Professor, Neurology
SchoolSchool of Medicine
Address675 Nelson Rising Lane, #317
San Francisco CA 94158
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    Other Positions
    Title(s)UCSF Weill Institute for Neurosciences

    Title(s)Director, Institute for Neurodegenerative Diseases

    Collapse Biography 
    Collapse Education and Training
    University of California, San FranciscoResidency School of Medicine
    Collapse Awards and Honors
    2009National Medal of Science
    1997Nobel Prize in Physiology or Medicine
    1994Albert Lasker Award for Basic Medical Research

    Collapse Overview 
    Collapse Overview
    Stanley B. Prusiner, MD, is a professor of neurology and biochemistry and director of the Institute for Neurodegenerative Diseases at UCSF. While at the university, Dr. Prusiner discovered an unprecedented class of pathogens that he named prions. Prions are infectious proteins that cause neurodegenerative diseases in animals and humans.

    Dr. Prusiner’s contributions to scientific research have been internationally recognized with numerous prizes including the Richard Lounsbery Award, the Albert Lasker Award and the Nobel Prize.

    Editor of 11 books and more than 500 research articles, Dr. Prusiner holds 50 issued or allowed United States patents, all of which are assigned to the University of California.

    He is a member of the National Academy of Sciences, the Institute of Medicine, the American Academy of Arts and Sciences and the American Philosophical Society, and is a foreign member of the Royal Society, London

    Dr. Prusiner received his undergraduate and medical training at the University of Pennsylvania and his postgraduate clinical training at UCSF.

    Collapse Research 
    Collapse Research Activities and Funding
    Towards Therapeutics for Neurodegenerative Diseases
    NIH R37AG031220Feb 15, 2008 - Jan 31, 2019
    Role: Principal Investigator
    Preventing Human Prion Diseases - Inactivation of Prions
    NIH R01AI064709Apr 1, 2006 - Mar 31, 2011
    Role: Principal Investigator
    Novel Therapeutics for Prion Diseases
    NIH P01AG021601Jun 1, 2003 - Feb 28, 2015
    Role: Principal Investigator
    Genetics of Prion Susceptibility in vitro
    NIH P01NS041997Sep 30, 2001 - Jun 30, 2013
    Role: Co-Investigator
    NIH C06RR016267Jun 15, 2001 - Jun 14, 2004
    Role: Principal Investigator
    NIH P41RR010888Jul 1, 1996 - Aug 9, 2012
    Role: Co-Investigator
    Molecular Pathogenesis of Age-Dependent CNS Regeneration
    NIH P01AG010770Apr 1, 1993 - Mar 31, 2016
    Role: Principal Investigator
    NIH R35AG008967May 1, 1990 - Apr 30, 1997
    Role: Principal Investigator
    NIH S10RR004691Mar 30, 1989 - Mar 29, 1990
    Role: Principal Investigator
    NIH P01NS022786Sep 1, 1985 - Jun 30, 2000
    Role: Principal Investigator
    NIH T32NS007219Apr 1, 1982 - Jun 30, 2003
    Role: Principal Investigator
    Bio-Organic Biomedical Mass Spectrometry Resource
    NIH P41RR001614Mar 1, 1982 - May 31, 2015
    Role: Co-Investigator
    Degenerative and Dementing Diseases of Aging
    NIH P01AG002132Jan 1, 1981 - Mar 31, 2025
    Role: Principal Investigator
    NIH/NINDS N01NS002328Feb 1, 1980 - Sep 30, 1986
    Role: Co-Principal Investigator
    NIH P01NS014069Dec 1, 1977 - Jun 30, 1997
    Role: Principal Investigator
    General Clinical Research Center
    NIH M01RR000079Dec 1, 1974 - Mar 31, 2009
    Role: Co-Investigator
    NIH P50NS014069Nov 30, 2002
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help. to make corrections and additions.
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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Guam ALS-PDC is a distinct double-prion disorder featuring both tau and A� prions. Proc Natl Acad Sci U S A. 2023 Mar 28; 120(13):e2220984120. Condello C, Ayers JI, Dalgard CL, Garcia Garcia MM, Rivera BM, Seeley WW, Perl DP, Prusiner SB. PMID: 36952379.
      View in: PubMed   Mentions:    Fields:    Translation:Humans
    2. Aβ and Tau Prions Causing Alzheimer's Disease. Methods Mol Biol. 2023; 2561:293-337. Condello C, Merz GE, Aoyagi A, DeGrado WF, Prusiner SB. PMID: 36399277.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimals
    3. Aβ and tau prions feature in the neuropathogenesis of Down syndrome. Proc Natl Acad Sci U S A. 2022 11 16; 119(46):e2212954119. Condello C, Maxwell AM, Castillo E, Aoyagi A, Graff C, Ingelsson M, Lannfelt L, Bird TD, Keene CD, Seeley WW, Perl DP, Head E, Prusiner SB. PMID: 36343257; PMCID: PMC9674250.
      View in: PubMed   Mentions: 2     Fields:    Translation:Humans
    4. Multiple system atrophy prions transmit neurological disease to mice expressing wild-type human α-synuclein. Acta Neuropathol. 2022 Oct; 144(4):677-690. Holec SAM, Lee J, Oehler A, Ooi FK, Mordes DA, Olson SH, Prusiner SB, Woerman AL. PMID: 36018376; PMCID: PMC9636591.
      View in: PubMed   Mentions:    Fields:    Translation:HumansAnimalsCells
    5. Trans-channel fluorescence learning improves high-content screening for Alzheimer's disease therapeutics. Nat Mach Intell. 2022 Jun; 4(6):583-595. Wong DR, Conrad J, Johnson N, Ayers J, Laeremans A, Lee JC, Lee J, Prusiner SB, Bandyopadhyay S, Butte AJ, Paras NA, Keiser MJ. PMID: 36276634; PMCID: PMC9585544.
      View in: PubMed   Mentions:
    6. Different α-synuclein prion strains cause dementia with Lewy bodies and multiple system atrophy. Proc Natl Acad Sci U S A. 2022 02 08; 119(6). Ayers JI, Lee J, Monteiro O, Woerman AL, Lazar AA, Condello C, Paras NA, Prusiner SB. PMID: 35115402; PMCID: PMC8833220.
      View in: PubMed   Mentions: 13     Fields:    Translation:HumansCells
    7. How an Infection of Sheep Revealed Prion Mechanisms in Alzheimer's Disease and Other Neurodegenerative Disorders. Int J Mol Sci. 2021 May 04; 22(9). Carlson GA, Prusiner SB. PMID: 34064393; PMCID: PMC8125442.
      View in: PubMed   Mentions: 14     Fields:    Translation:HumansAnimalsCells
    8. Water-Soluble Iridium Photoredox Catalyst for the Trifluoromethylation of Biomolecule Substrates in Phosphate Buffered Saline Solvent. Org Lett. 2021 05 21; 23(10):3823-3827. Nguyen TH, O'Brien CJ, Tran MLN, Olson SH, Settineri NS, Prusiner SB, Paras NA, Conrad J. PMID: 33929208.
      View in: PubMed   Mentions: 3     Fields:    Translation:Cells
    9. Tau aggregates are RNA-protein assemblies that mislocalize multiple nuclear speckle components. Neuron. 2021 05 19; 109(10):1675-1691.e9. Lester E, Ooi FK, Bakkar N, Ayers J, Woerman AL, Wheeler J, Bowser R, Carlson GA, Prusiner SB, Parker R. PMID: 33848474; PMCID: PMC8141031.
      View in: PubMed   Mentions: 32     Fields:    Translation:HumansAnimalsCells
    10. Silver Benzoate Facilitates the Copper-Catalyzed C-N Coupling of Iodoazoles with Aromatic Nitrogen Heterocycles. ACS Omega. 2021 Apr 13; 6(14):9804-9812. Lozano C, Ramirez C, Sin N, Viart HM, Prusiner SB, Paras NA, Conrad J. PMID: 33869960; PMCID: PMC8047741.
      View in: PubMed   Mentions:
    11. Prion biology: implications for Alzheimer's disease therapeutics. Lancet Neurol. 2020 10; 19(10):802-803. Condello C, DeGrado WF, Prusiner SB. PMID: 32949533.
      View in: PubMed   Mentions: 5     Fields:    Translation:Humans
    12. Expanding spectrum of prion diseases. Emerg Top Life Sci. 2020 09 08; 4(2):155-167. Ayers JI, Paras NA, Prusiner SB. PMID: 32803268.
      View in: PubMed   Mentions: 16     Fields:    Translation:HumansAnimalsCells
    13. Prion protein - mediator of toxicity in multiple proteinopathies. Nat Rev Neurol. 2020 04; 16(4):187-188. Ayers JI, Prusiner SB. PMID: 32123368.
      View in: PubMed   Mentions: 3     Fields:    
    14. Kinetics of α-synuclein prions preceding neuropathological inclusions in multiple system atrophy. PLoS Pathog. 2020 02; 16(2):e1008222. Woerman AL, Patel S, Kazmi SA, Oehler A, Lee J, Mordes DA, Olson SH, Prusiner SB. PMID: 32017806; PMCID: PMC6999861.
      View in: PubMed   Mentions: 12     Fields:    Translation:HumansAnimals
    15. Discovery of 4-Piperazine Isoquinoline Derivatives as Potent and Brain-Permeable Tau Prion Inhibitors with CDK8 Activity. ACS Med Chem Lett. 2020 Feb 13; 11(2):127-132. Grandjean JM, Jiu AY, West JW, Aoyagi A, Droege DG, Elepano M, Hirasawa M, Hirouchi M, Murakami R, Lee J, Sasaki K, Hirano S, Ohyama T, Tang BC, Vaz RJ, Inoue M, Olson SH, Prusiner SB, Conrad J, Paras NA. PMID: 32071678; PMCID: PMC7025388.
      View in: PubMed   Mentions: 2  
    16. Replication of multiple system atrophy prions in primary astrocyte cultures from transgenic mice expressing human α-synuclein. Acta Neuropathol Commun. 2019 05 20; 7(1):81. Krejciova Z, Carlson GA, Giles K, Prusiner SB. PMID: 31109379; PMCID: PMC6526619.
      View in: PubMed   Mentions: 6     Fields:    Translation:HumansAnimalsCells
    17. Aβ and tau prion-like activities decline with longevity in the Alzheimer's disease human brain. Sci Transl Med. 2019 05 01; 11(490). Aoyagi A, Condello C, Stöhr J, Yue W, Rivera BM, Lee JC, Woerman AL, Halliday G, van Duinen S, Ingelsson M, Lannfelt L, Graff C, Bird TD, Keene CD, Seeley WW, DeGrado WF, Prusiner SB. PMID: 31043574; PMCID: PMC6640844.
      View in: PubMed   Mentions: 49     Fields:    Translation:HumansAnimalsCells
    18. Multiple system atrophy prions retain strain specificity after serial propagation in two different Tg(SNCA*A53T) mouse lines. Acta Neuropathol. 2019 03; 137(3):437-454. Woerman AL, Oehler A, Kazmi SA, Lee J, Halliday GM, Middleton LT, Gentleman SM, Mordes DA, Spina S, Grinberg LT, Olson SH, Prusiner SB. PMID: 30690664; PMCID: PMC6454887.
      View in: PubMed   Mentions: 43     Fields:    Translation:HumansAnimals
    19. α-Synuclein: Multiple System Atrophy Prions. Cold Spring Harb Perspect Med. 2018 07 02; 8(7). Woerman AL, Watts JC, Aoyagi A, Giles K, Middleton LT, Prusiner SB. PMID: 28213437; PMCID: PMC5561534.
      View in: PubMed   Mentions: 39     Fields:    Translation:HumansAnimalsCells
    20. Citations must default to the online publication date. Nature. 2018 06; 558(7711):519. Keller M, Prusiner S. PMID: 29950625.
      View in: PubMed   Mentions:    Fields:    
    21. β-Amyloid Prions and the Pathobiology of Alzheimer's Disease. Cold Spring Harb Perspect Med. 2018 05 01; 8(5). Watts JC, Prusiner SB. PMID: 28193770; PMCID: PMC5554751.
      View in: PubMed   Mentions: 37     Fields:    Translation:HumansAnimals
    22. A long-lived Aβ oligomer resistant to fibrillization. Biopolymers. 2018 Aug; 109(8):e23096. Nick M, Wu Y, Schmidt NW, Prusiner SB, Stöhr J, DeGrado WF. PMID: 29319162; PMCID: PMC6039285.
      View in: PubMed   Mentions: 7     Fields:    Translation:Humans
    23. Structural heterogeneity and intersubject variability of Aβ in familial and sporadic Alzheimer's disease. Proc Natl Acad Sci U S A. 2018 01 23; 115(4):E782-E791. Condello C, Lemmin T, Stöhr J, Nick M, Wu Y, Maxwell AM, Watts JC, Caro CD, Oehler A, Keene CD, Bird TD, van Duinen SG, Lannfelt L, Ingelsson M, Graff C, Giles K, DeGrado WF, Prusiner SB. PMID: 29311311; PMCID: PMC5789926.
      View in: PubMed   Mentions: 57     Fields:    Translation:AnimalsCells
    24. Familial Parkinson's point mutation abolishes multiple system atrophy prion replication. Proc Natl Acad Sci U S A. 2018 01 09; 115(2):409-414. Woerman AL, Kazmi SA, Patel S, Aoyagi A, Oehler A, Widjaja K, Mordes DA, Olson SH, Prusiner SB. PMID: 29279394; PMCID: PMC5777081.
      View in: PubMed   Mentions: 21     Fields:    Translation:HumansAnimalsCells
    25. Evidence for sortilin modulating regional accumulation of human tau prions in transgenic mice. Proc Natl Acad Sci U S A. 2017 12 19; 114(51):E11029-E11036. Johnson NR, Condello C, Guan S, Oehler A, Becker J, Gavidia M, Carlson GA, Giles K, Prusiner SB. PMID: 29203673; PMCID: PMC5754811.
      View in: PubMed   Mentions: 10     Fields:    Translation:HumansAnimalsCells
    26. Kinetics of Human Mutant Tau Prion Formation in the Brains of 2 Transgenic Mouse Lines. JAMA Neurol. 2017 12 01; 74(12):1464-1472. Woerman AL, Patel S, Kazmi SA, Oehler A, Freyman Y, Espiritu L, Cotter R, Castaneda JA, Olson SH, Prusiner SB. PMID: 29059326; PMCID: PMC5822201.
      View in: PubMed   Mentions: 19     Fields:    Translation:HumansAnimals
    27. A novel vector for transgenesis in the rat CNS. Acta Neuropathol Commun. 2017 Nov 21; 5(1):84. Lopez TP, Giles K, Dugger BN, Oehler A, Condello C, Krejciova Z, Castaneda JA, Carlson GA, Prusiner SB. PMID: 29157304; PMCID: PMC5697436.
      View in: PubMed   Mentions:    Fields:    Translation:AnimalsCells
    28. Experimental Models of Inherited PrP Prion Diseases. Cold Spring Harb Perspect Med. 2017 Nov 01; 7(11). Watts JC, Prusiner SB. PMID: 28096244; PMCID: PMC5513788.
      View in: PubMed   Mentions: 10     Fields:    Translation:HumansAnimals
    29. MSA prions exhibit remarkable stability and resistance to inactivation. Acta Neuropathol. 2018 01; 135(1):49-63. Woerman AL, Kazmi SA, Patel S, Freyman Y, Oehler A, Aoyagi A, Mordes DA, Halliday GM, Middleton LT, Gentleman SM, Olson SH, Prusiner SB. PMID: 28849371; PMCID: PMC5756500.
      View in: PubMed   Mentions: 46     Fields:    Translation:HumansAnimalsCells
    30. Bioassays and Inactivation of Prions. Cold Spring Harb Perspect Biol. 2017 Aug 01; 9(8). Giles K, Woerman AL, Berry DB, Prusiner SB. PMID: 28246183; PMCID: PMC5538415.
      View in: PubMed   Mentions: 19     Fields:    Translation:HumansAnimals
    31. Developing Therapeutics for PrP Prion Diseases. Cold Spring Harb Perspect Med. 2017 Apr 03; 7(4). Giles K, Olson SH, Prusiner SB. PMID: 28096242; PMCID: PMC5378016.
      View in: PubMed   Mentions: 27     Fields:    Translation:HumansAnimalsCells
    32. A 31-residue peptide induces aggregation of tau's microtubule-binding region in cells. Nat Chem. 2017 09; 9(9):874-881. Stöhr J, Wu H, Nick M, Wu Y, Bhate M, Condello C, Johnson N, Rodgers J, Lemmin T, Acharya S, Becker J, Robinson K, Kelly MJS, Gai F, Stubbs G, Prusiner SB, DeGrado WF. PMID: 28837163; PMCID: PMC5759337.
      View in: PubMed   Mentions: 23     Fields:    Translation:HumansCells
    33. Tau prions from Alzheimer's disease and chronic traumatic encephalopathy patients propagate in cultured cells. Proc Natl Acad Sci U S A. 2016 12 13; 113(50):E8187-E8196. Woerman AL, Aoyagi A, Patel S, Kazmi SA, Lobach I, Grinberg LT, McKee AC, Seeley WW, Olson SH, Prusiner SB. PMID: 27911827; PMCID: PMC5167200.
      View in: PubMed   Mentions: 82     Fields:    Translation:HumansCells
    34. Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions. J Virol. 2016 11 01; 90(21):9558-9569. Watts JC, Giles K, Saltzberg DJ, Dugger BN, Patel S, Oehler A, Bhardwaj S, Sali A, Prusiner SB. PMID: 27440899; PMCID: PMC5068510.
      View in: PubMed   Mentions: 2     Fields:    Translation:HumansAnimals
    35. Structural Polymorphism of Alzheimer's β-Amyloid Fibrils as Controlled by an E22 Switch: A Solid-State NMR Study. J Am Chem Soc. 2016 08 10; 138(31):9840-52. Elkins MR, Wang T, Nick M, Jo H, Lemmin T, Prusiner SB, DeGrado WF, Stöhr J, Hong M. PMID: 27414264; PMCID: PMC5149419.
      View in: PubMed   Mentions: 37     Fields:    Translation:HumansCells
    36. FoxO3 regulates neuronal reprogramming of cells from postnatal and aging mice. Proc Natl Acad Sci U S A. 2016 07 26; 113(30):8514-9. Ahlenius H, Chanda S, Webb AE, Yousif I, Karmazin J, Prusiner SB, Brunet A, Südhof TC, Wernig M. PMID: 27402759; PMCID: PMC4968717.
      View in: PubMed   Mentions: 10     Fields:    Translation:AnimalsCells
    37. Towards authentic transgenic mouse models of heritable PrP prion diseases. Acta Neuropathol. 2016 10; 132(4):593-610. Watts JC, Giles K, Bourkas ME, Patel S, Oehler A, Gavidia M, Bhardwaj S, Lee J, Prusiner SB. PMID: 27350609; PMCID: PMC5152593.
      View in: PubMed   Mentions: 25     Fields:    Translation:Animals
    38. Optimization of Aryl Amides that Extend Survival in Prion-Infected Mice. J Pharmacol Exp Ther. 2016 09; 358(3):537-47. Giles K, Berry DB, Condello C, Dugger BN, Li Z, Oehler A, Bhardwaj S, Elepano M, Guan S, Silber BM, Olson SH, Prusiner SB. PMID: 27317802; PMCID: PMC4998675.
      View in: PubMed   Mentions: 14     Fields:    Translation:Animals
    39. Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism. Proc Natl Acad Sci U S A. 2015 Sep 22; 112(38):E5308-17. Prusiner SB, Woerman AL, Mordes DA, Watts JC, Rampersaud R, Berry DB, Patel S, Oehler A, Lowe JK, Kravitz SN, Geschwind DH, Glidden DV, Halliday GM, Middleton LT, Gentleman SM, Grinberg LT, Giles K. PMID: 26324905; PMCID: PMC4586853.
      View in: PubMed   Mentions: 342     Fields:    Translation:HumansAnimalsCells
    40. Modulation of Creutzfeldt-Jakob disease prion propagation by the A224V mutation. Ann Neurol. 2015 Oct; 78(4):540-53. Watts JC, Giles K, Serban A, Patel S, Oehler A, Bhardwaj S, Guan S, Greicius MD, Miller BL, DeArmond SJ, Geschwind MD, Prusiner SB. PMID: 26094969; PMCID: PMC4711268.
      View in: PubMed   Mentions: 14     Fields:    Translation:HumansAnimals
    41. Prion Protein-Antibody Complexes Characterized by Chromatography-Coupled Small-Angle X-Ray Scattering. Biophys J. 2015 Aug 18; 109(4):793-805. Carter L, Kim SJ, Schneidman-Duhovny D, Stöhr J, Poncet-Montange G, Weiss TM, Tsuruta H, Prusiner SB, Sali A. PMID: 26287631; PMCID: PMC4547163.
      View in: PubMed   Mentions: 19     Fields:    Translation:AnimalsCells
    42. Propagation of prions causing synucleinopathies in cultured cells. Proc Natl Acad Sci U S A. 2015 Sep 01; 112(35):E4949-58. Woerman AL, Stöhr J, Aoyagi A, Rampersaud R, Krejciova Z, Watts JC, Ohyama T, Patel S, Widjaja K, Oehler A, Sanders DW, Diamond MI, Seeley WW, Middleton LT, Gentleman SM, Mordes DA, Südhof TC, Giles K, Prusiner SB. PMID: 26286986; PMCID: PMC4568231.
      View in: PubMed   Mentions: 120     Fields:    Translation:HumansAnimalsCells
    43. Different 2-Aminothiazole Therapeutics Produce Distinct Patterns of Scrapie Prion Neuropathology in Mouse Brains. J Pharmacol Exp Ther. 2015 Oct; 355(1):2-12. Giles K, Berry DB, Condello C, Hawley RC, Gallardo-Godoy A, Bryant C, Oehler A, Elepano M, Bhardwaj S, Patel S, Silber BM, Guan S, DeArmond SJ, Renslo AR, Prusiner SB. PMID: 26224882; PMCID: PMC4576665.
      View in: PubMed   Mentions: 23     Fields:    Translation:HumansAnimals
    44. Use of a 2-aminothiazole to Treat Chronic Wasting Disease in Transgenic Mice. J Infect Dis. 2015 Jul 15; 212 Suppl 1:S17-25. Berry D, Giles K, Oehler A, Bhardwaj S, DeArmond SJ, Prusiner SB. PMID: 26116725; PMCID: PMC4551108.
      View in: PubMed   Mentions: 13     Fields:    Translation:AnimalsCells
    45. Structural studies of truncated forms of the prion protein PrP. Biophys J. 2015 Mar 24; 108(6):1548-1554. Wan W, Wille H, Stöhr J, Kendall A, Bian W, McDonald M, Tiggelaar S, Watts JC, Prusiner SB, Stubbs G. PMID: 25809267; PMCID: PMC4375555.
      View in: PubMed   Mentions: 15     Fields:    Translation:HumansAnimalsCells
    46. Mechanism of scrapie prion precipitation with phosphotungstate anions. ACS Chem Biol. 2015 May 15; 10(5):1269-77. Levine DJ, Stöhr J, Falese LE, Ollesch J, Wille H, Prusiner SB, Long JR. PMID: 25695325; PMCID: PMC4437617.
      View in: PubMed   Mentions: 16     Fields:    Translation:Animals
    47. Distinct synthetic Aβ prion strains producing different amyloid deposits in bigenic mice. Proc Natl Acad Sci U S A. 2014 Jul 15; 111(28):10329-34. Stöhr J, Condello C, Watts JC, Bloch L, Oehler A, Nick M, DeArmond SJ, Giles K, DeGrado WF, Prusiner SB. PMID: 24982137; PMCID: PMC4104853.
      View in: PubMed   Mentions: 89     Fields:    Translation:HumansAnimals
    48. Serial propagation of distinct strains of Aβ prions from Alzheimer's disease patients. Proc Natl Acad Sci U S A. 2014 Jul 15; 111(28):10323-8. Watts JC, Condello C, Stöhr J, Oehler A, Lee J, DeArmond SJ, Lannfelt L, Ingelsson M, Giles K, Prusiner SB. PMID: 24982139; PMCID: PMC4104857.
      View in: PubMed   Mentions: 153     Fields:    Translation:HumansAnimals
    49. Mouse models for studying the formation and propagation of prions. J Biol Chem. 2014 Jul 18; 289(29):19841-9. Watts JC, Prusiner SB. PMID: 24860095; PMCID: PMC4106304.
      View in: PubMed   Mentions: 50     Fields:    Translation:HumansAnimals
    50. Evidence that bank vole PrP is a universal acceptor for prions. PLoS Pathog. 2014 Apr; 10(4):e1003990. Watts JC, Giles K, Patel S, Oehler A, DeArmond SJ, Prusiner SB. PMID: 24699458; PMCID: PMC3974871.
      View in: PubMed   Mentions: 64     Fields:    Translation:HumansAnimals
    51. Novel compounds lowering the cellular isoform of the human prion protein in cultured human cells. Bioorg Med Chem. 2014 Mar 15; 22(6):1960-72. Silber BM, Gever JR, Rao S, Li Z, Renslo AR, Widjaja K, Wong C, Giles K, Freyman Y, Elepano M, Irwin JJ, Jacobson MP, Prusiner SB. PMID: 24530226; PMCID: PMC3984052.
      View in: PubMed   Mentions: 10     Fields:    Translation:HumansAnimalsCells
    52. Transmission of multiple system atrophy prions to transgenic mice. Proc Natl Acad Sci U S A. 2013 Nov 26; 110(48):19555-60. Watts JC, Giles K, Oehler A, Middleton L, Dexter DT, Gentleman SM, DeArmond SJ, Prusiner SB. PMID: 24218576; PMCID: PMC3845125.
      View in: PubMed   Mentions: 210     Fields:    Translation:HumansAnimalsPHPublic Health
    53. Drug resistance confounding prion therapeutics. Proc Natl Acad Sci U S A. 2013 Oct 29; 110(44):E4160-9. Berry DB, Lu D, Geva M, Watts JC, Bhardwaj S, Oehler A, Renslo AR, DeArmond SJ, Prusiner SB, Giles K. PMID: 24128760; PMCID: PMC3816483.
      View in: PubMed   Mentions: 65     Fields:    Translation:HumansAnimalsCells
    54. Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease. Neurology. 2013 Dec 03; 81(23):2015-23. Geschwind MD, Kuo AL, Wong KS, Haman A, Devereux G, Raudabaugh BJ, Johnson DY, Torres-Chae CC, Finley R, Garcia P, Thai JN, Cheng HQ, Neuhaus JM, Forner SA, Duncan JL, Possin KL, Dearmond SJ, Prusiner SB, Miller BL. PMID: 24122181; PMCID: PMC4211922.
      View in: PubMed   Mentions: 41     Fields:    Translation:Humans
    55. Antiprion compounds that reduce PrP(Sc) levels in dividing and stationary-phase cells. Bioorg Med Chem. 2013 Dec 15; 21(24):7999-8012. Silber BM, Gever JR, Li Z, Gallardo-Godoy A, Renslo AR, Widjaja K, Irwin JJ, Rao S, Jacobson MP, Ghaemmaghami S, Prusiner SB. PMID: 24183589; PMCID: PMC3984054.
      View in: PubMed   Mentions: 6     Fields:    Translation:AnimalsCells
    56. A high-throughput screening assay for determining cellular levels of total tau protein. Curr Alzheimer Res. 2013 Sep; 10(7):679-87. Dehdashti SJ, Zheng W, Gever JR, Wilhelm R, Nguyen DT, Sittampalam G, McKew JC, Austin CP, Prusiner SB. PMID: 23905996; PMCID: PMC4010324.
      View in: PubMed   Mentions: 10     Fields:    Translation:HumansAnimalsCells
    57. Biaryl amides and hydrazones as therapeutics for prion disease in transgenic mice. J Pharmacol Exp Ther. 2013 Nov; 347(2):325-38. Lu D, Giles K, Li Z, Rao S, Dolghih E, Gever JR, Geva M, Elepano ML, Oehler A, Bryant C, Renslo AR, Jacobson MP, Dearmond SJ, Silber BM, Prusiner SB. PMID: 23965382; PMCID: PMC3807058.
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    58. Optimization of Arylamides as Novel, Potent and Brain-penetrant Antiprion Lead Compounds. ACS Med Chem Lett. 2013 Jul 11; 4(7):647-650. Li Z, Rao S, Gever JR, Widjaja K, Prusiner SB, Silber BM. PMID: 23977416; PMCID: PMC3746989.
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    59. Plasma membrane invaginations containing clusters of full-length PrPSc are an early form of prion-associated neuropathology in vivo. Neurobiol Aging. 2013 Jun; 34(6):1621-31. Godsave SF, Wille H, Pierson J, Prusiner SB, Peters PJ. PMID: 23481568; PMCID: PMC3596824.
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    60. Discovery and Preliminary SAR of Arylpiperazines as Novel, Brainpenetrant Antiprion Compounds. ACS Med Chem Lett. 2013 Apr 11; 4(4):397-401. Li Z, Gever J, Rao S, Widjaja K, Prusiner SB, Silber BM. PMID: 23847718; PMCID: PMC3704178.
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    61. 2-Aminothiazoles with improved pharmacotherapeutic properties for treatment of prion disease. ChemMedChem. 2013 May; 8(5):847-57. Li Z, Silber BM, Rao S, Gever JR, Bryant C, Gallardo-Godoy A, Dolghih E, Widjaja K, Elepano M, Jacobson MP, Prusiner SB, Renslo AR. PMID: 23509039; PMCID: PMC3984044.
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    62. Convergent replication of mouse synthetic prion strains. Am J Pathol. 2013 Mar; 182(3):866-74. Ghaemmaghami S, Colby DW, Nguyen HO, Hayashi S, Oehler A, DeArmond SJ, Prusiner SB. PMID: 23438476; PMCID: PMC3586687.
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    63. Pharmacokinetics and metabolism of 2-aminothiazoles with antiprion activity in mice. Pharm Res. 2013 Apr; 30(4):932-50. Silber BM, Rao S, Fife KL, Gallardo-Godoy A, Renslo AR, Dalvie DK, Giles K, Freyman Y, Elepano M, Gever JR, Li Z, Jacobson MP, Huang Y, Benet LZ, Prusiner SB. PMID: 23417511; PMCID: PMC3640342.
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    64. Biology and genetics of prions causing neurodegeneration. Annu Rev Genet. 2013; 47:601-23. Prusiner SB. PMID: 24274755; PMCID: PMC4010318.
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    65. Chimeric elk/mouse prion proteins in transgenic mice. J Gen Virol. 2013 Feb; 94(Pt 2):443-452. Tamgüney G, Giles K, Oehler A, Johnson NL, DeArmond SJ, Prusiner SB. PMID: 23100369; PMCID: PMC3709613.
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    66. Novel epitopes identified by anti-PrP monoclonal antibodies produced following immunization of Prnp0/0 Balb/cJ mice with purified scrapie prions. Hybridoma (Larchmt). 2012 Oct; 31(5):314-24. Stanker LH, Scotcher MC, Lin A, McGarvey J, Prusiner SB, Hnasko R. PMID: 23098297; PMCID: PMC3482378.
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    67. Pharmacokinetics of quinacrine efflux from mouse brain via the P-glycoprotein efflux transporter. PLoS One. 2012; 7(7):e39112. Ahn M, Ghaemmaghami S, Huang Y, Phuan PW, May BC, Giles K, DeArmond SJ, Prusiner SB. PMID: 22768295; PMCID: PMC3388068.
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    68. Cell biology. A unifying role for prions in neurodegenerative diseases. Science. 2012 Jun 22; 336(6088):1511-3. Prusiner SB. PMID: 22723400; PMCID: PMC3942086.
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    69. Purified and synthetic Alzheimer's amyloid beta (Aβ) prions. Proc Natl Acad Sci U S A. 2012 Jul 03; 109(27):11025-30. Stöhr J, Watts JC, Mensinger ZL, Oehler A, Grillo SK, DeArmond SJ, Prusiner SB, Giles K. PMID: 22711819; PMCID: PMC3390876.
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    70. Degradation of fungal prion HET-s(218-289) induces formation of a generic amyloid fold. Biophys J. 2012 May 16; 102(10):2339-44. Wan W, Wille H, Stöhr J, Baxa U, Prusiner SB, Stubbs G. PMID: 22677387; PMCID: PMC3353098.
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    71. Compartment modeling for mammalian protein turnover studies by stable isotope metabolic labeling. Anal Chem. 2012 May 01; 84(9):4014-21. Guan S, Price JC, Ghaemmaghami S, Prusiner SB, Burlingame AL. PMID: 22444387; PMCID: PMC3923578.
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    72. Identification of I137M and other mutations that modulate incubation periods for two human prion strains. J Virol. 2012 Jun; 86(11):6033-41. Giles K, De Nicola GF, Patel S, Glidden DV, Korth C, Oehler A, DeArmond SJ, Prusiner SB. PMID: 22438549; PMCID: PMC3372217.
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    73. Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein. Proc Natl Acad Sci U S A. 2012 Feb 28; 109(9):3498-503. Watts JC, Giles K, Stöhr J, Oehler A, Bhardwaj S, Grillo SK, Patel S, DeArmond SJ, Prusiner SB. PMID: 22331873; PMCID: PMC3295307.
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    74. Intracerebral Infusion of Antisense Oligonucleotides Into Prion-infected Mice. Mol Ther Nucleic Acids. 2012 Feb 07; 1:e9. Nazor Friberg K, Hung G, Wancewicz E, Giles K, Black C, Freier S, Bennett F, Dearmond SJ, Freyman Y, Lessard P, Ghaemmaghami S, Prusiner SB. PMID: 23344724; PMCID: PMC3381600.
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    75. Salivary prions in sheep and deer. Prion. 2012 Jan-Mar; 6(1):52-61. Tamgüney G, Richt JA, Hamir AN, Greenlee JJ, Miller MW, Wolfe LL, Sirochman TM, Young AJ, Glidden DV, Johnson NL, Giles K, DeArmond SJ, Prusiner SB. PMID: 22453179; PMCID: PMC3338966.
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    76. Prion uptake in the gut: identification of the first uptake and replication sites. PLoS Pathog. 2011 Dec; 7(12):e1002449. Kujala P, Raymond CR, Romeijn M, Godsave SF, van Kasteren SI, Wille H, Prusiner SB, Mabbott NA, Peters PJ. PMID: 22216002; PMCID: PMC3245311.
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    77. Spontaneous generation of anchorless prions in transgenic mice. Proc Natl Acad Sci U S A. 2011 Dec 27; 108(52):21223-8. Stöhr J, Watts JC, Legname G, Oehler A, Lemus A, Nguyen HO, Sussman J, Wille H, DeArmond SJ, Prusiner SB, Giles K. PMID: 22160704; PMCID: PMC3248514.
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    78. Protease-resistant prions selectively decrease Shadoo protein. PLoS Pathog. 2011 Nov; 7(11):e1002382. Watts JC, Stöhr J, Bhardwaj S, Wille H, Oehler A, Dearmond SJ, Giles K, Prusiner SB. PMID: 22163178; PMCID: PMC3219722.
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    79. De novo generation of prion strains. Nat Rev Microbiol. 2011 Sep 26; 9(11):771-7. Colby DW, Prusiner SB. PMID: 21947062; PMCID: PMC3924856.
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    80. A data processing pipeline for mammalian proteome dynamics studies using stable isotope metabolic labeling. Mol Cell Proteomics. 2011 Dec; 10(12):M111.010728. Guan S, Price JC, Prusiner SB, Ghaemmaghami S, Burlingame AL. PMID: 21937731; PMCID: PMC3237081.
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    81. Conformational transformation and selection of synthetic prion strains. J Mol Biol. 2011 Oct 28; 413(3):527-42. Ghaemmaghami S, Watts JC, Nguyen HO, Hayashi S, DeArmond SJ, Prusiner SB. PMID: 21839745; PMCID: PMC3195964.
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    82. Conserved properties of human and bovine prion strains on transmission to guinea pigs. Lab Invest. 2011 Sep; 91(9):1326-36. Safar JG, Giles K, Lessard P, Letessier F, Patel S, Serban A, Dearmond SJ, Prusiner SB. PMID: 21727894; PMCID: PMC3164925.
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    83. A survey of antiprion compounds reveals the prevalence of non-PrP molecular targets. J Biol Chem. 2011 Aug 05; 286(31):27718-28. Poncet-Montange G, St Martin SJ, Bogatova OV, Prusiner SB, Shoichet BK, Ghaemmaghami S. PMID: 21610081; PMCID: PMC3149362.
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    84. Bioluminescence imaging of Abeta deposition in bigenic mouse models of Alzheimer's disease. Proc Natl Acad Sci U S A. 2011 Feb 08; 108(6):2528-33. Watts JC, Giles K, Grillo SK, Lemus A, DeArmond SJ, Prusiner SB. PMID: 21262831; PMCID: PMC3038719.
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    85. 2-Aminothiazoles as therapeutic leads for prion diseases. J Med Chem. 2011 Feb 24; 54(4):1010-21. Gallardo-Godoy A, Gever J, Fife KL, Silber BM, Prusiner SB, Renslo AR. PMID: 21247166; PMCID: PMC3041857.
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    86. Prions. Cold Spring Harb Perspect Biol. 2011 Jan 01; 3(1):a006833. Colby DW, Prusiner SB. PMID: 21421910; PMCID: PMC3003464.
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    87. Analysis of proteome dynamics in the mouse brain. Proc Natl Acad Sci U S A. 2010 Aug 10; 107(32):14508-13. Price JC, Guan S, Burlingame A, Prusiner SB, Ghaemmaghami S. PMID: 20699386; PMCID: PMC2922600.
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    88. Human prion strain selection in transgenic mice. Ann Neurol. 2010 Aug; 68(2):151-61. Giles K, Glidden DV, Patel S, Korth C, Groth D, Lemus A, DeArmond SJ, Prusiner SB. PMID: 20695008; PMCID: PMC2935907.
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    89. Conformation-dependent high-affinity monoclonal antibodies to prion proteins. J Immunol. 2010 Jul 01; 185(1):729-37. Stanker LH, Serban AV, Cleveland E, Hnasko R, Lemus A, Safar J, DeArmond SJ, Prusiner SB. PMID: 20530267; PMCID: PMC2957185.
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    90. Colloid formation by drugs in simulated intestinal fluid. J Med Chem. 2010 May 27; 53(10):4259-65. Doak AK, Wille H, Prusiner SB, Shoichet BK. PMID: 20426472; PMCID: PMC2874266.
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    91. Generation of antisera to purified prions in lipid rafts. Prion. 2010 Apr-Jun; 4(2):94-104. Hnasko R, Serban AV, Carlson G, Prusiner SB, Stanker LH. PMID: 20647769; PMCID: PMC2933057.
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    92. Protease-sensitive synthetic prions. PLoS Pathog. 2010 Jan 22; 6(1):e1000736. Colby DW, Wain R, Baskakov IV, Legname G, Palmer CG, Nguyen HO, Lemus A, Cohen FE, DeArmond SJ, Prusiner SB. PMID: 20107515; PMCID: PMC2809756.
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    93. Discovery of 2-aminothiazoles as potent antiprion compounds. J Virol. 2010 Apr; 84(7):3408-12. Ghaemmaghami S, May BC, Renslo AR, Prusiner SB. PMID: 20032192; PMCID: PMC2838138.
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    94. Chemical induction of misfolded prion protein conformers in cell culture. J Biol Chem. 2010 Apr 02; 285(14):10415-23. Ghaemmaghami S, Ullman J, Ahn M, St Martin S, Prusiner SB. PMID: 19955177; PMCID: PMC2856248.
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    95. Continuous quinacrine treatment results in the formation of drug-resistant prions. PLoS Pathog. 2009 Nov; 5(11):e1000673. Ghaemmaghami S, Ahn M, Lessard P, Giles K, Legname G, DeArmond SJ, Prusiner SB. PMID: 19956709; PMCID: PMC2777304.
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    96. Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci U S A. 2009 Dec 01; 106(48):20417-22. Colby DW, Giles K, Legname G, Wille H, Baskakov IV, DeArmond SJ, Prusiner SB. PMID: 19915150; PMCID: PMC2787151.
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    97. Natural and synthetic prion structure from X-ray fiber diffraction. Proc Natl Acad Sci U S A. 2009 Oct 06; 106(40):16990-5. Wille H, Bian W, McDonald M, Kendall A, Colby DW, Bloch L, Ollesch J, Borovinskiy AL, Cohen FE, Prusiner SB, Stubbs G. PMID: 19805070; PMCID: PMC2761340.
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    98. Asymptomatic deer excrete infectious prions in faeces. Nature. 2009 Sep 24; 461(7263):529-32. Tamgüney G, Miller MW, Wolfe LL, Sirochman TM, Glidden DV, Palmer C, Lemus A, DeArmond SJ, Prusiner SB. PMID: 19741608; PMCID: PMC3186440.
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    99. Prion proteins with pathogenic and protective mutations show similar structure and dynamics. Biochemistry. 2009 Sep 01; 48(34):8120-8. Bae SH, Legname G, Serban A, Prusiner SB, Wright PE, Dyson HJ. PMID: 19618915; PMCID: PMC2762478.
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    100. Measuring prions by bioluminescence imaging. Proc Natl Acad Sci U S A. 2009 Sep 01; 106(35):15002-6. Tamgüney G, Francis KP, Giles K, Lemus A, DeArmond SJ, Prusiner SB. PMID: 19706444; PMCID: PMC2736416.
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    101. Is Parkinson's disease a prion disorder? Proc Natl Acad Sci U S A. 2009 Aug 04; 106(31):12571-2. Olanow CW, Prusiner SB. PMID: 19666621; PMCID: PMC2722298.
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    102. Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease. Lab Invest. 2009 Jun; 89(6):624-35. Choi EM, Geschwind MD, Deering C, Pomeroy K, Kuo A, Miller BL, Safar JG, Prusiner SB. PMID: 19434060; PMCID: PMC2786774.
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    103. Development of antibody fragments for immunotherapy of prion diseases. Biochem J. 2009 Mar 15; 418(3):507-15. Campana V, Zentilin L, Mirabile I, Kranjc A, Casanova P, Giacca M, Prusiner SB, Legname G, Zurzolo C. PMID: 19000036.
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    104. Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion protein. J Gen Virol. 2009 Apr; 90(Pt 4):1035-1047. Tamgüney G, Miller MW, Giles K, Lemus A, Glidden DV, DeArmond SJ, Prusiner SB. PMID: 19264659; PMCID: PMC2804060.
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    105. Surface charge of polyoxometalates modulates polymerization of the scrapie prion protein. Proc Natl Acad Sci U S A. 2009 Mar 10; 106(10):3740-5. Wille H, Shanmugam M, Murugesu M, Ollesch J, Stubbs G, Long JR, Safar JG, Prusiner SB. PMID: 19223590; PMCID: PMC2656150.
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    106. Cryo-immunogold electron microscopy for prions: toward identification of a conversion site. J Neurosci. 2008 Nov 19; 28(47):12489-99. Godsave SF, Wille H, Kujala P, Latawiec D, DeArmond SJ, Serban A, Prusiner SB, Peters PJ. PMID: 19020041; PMCID: PMC2796247.
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    107. Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLoS Pathog. 2008 Nov; 4(11):e1000206. Giles K, Glidden DV, Beckwith R, Seoanes R, Peretz D, DeArmond SJ, Prusiner SB. PMID: 19008948; PMCID: PMC2576443.
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    108. A gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains. Proc Natl Acad Sci U S A. 2008 Jul 29; 105(30):10595-600. Spilman P, Lessard P, Sattavat M, Bush C, Tousseyn T, Huang EJ, Giles K, Golde T, Das P, Fauq A, Prusiner SB, Dearmond SJ. PMID: 18647832; PMCID: PMC2492499.
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    109. Transmission and detection of prions in feces. J Infect Dis. 2008 Jul 01; 198(1):81-9. Safar JG, Lessard P, Tamgüney G, Freyman Y, Deering C, Letessier F, Dearmond SJ, Prusiner SB. PMID: 18505383; PMCID: PMC2803675.
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    110. Genes contributing to prion pathogenesis. J Gen Virol. 2008 Jul; 89(Pt 7):1777-1788. Tamgüney G, Giles K, Glidden DV, Lessard P, Wille H, Tremblay P, Groth DF, Yehiely F, Korth C, Moore RC, Tatzelt J, Rubinstein E, Boucheix C, Yang X, Stanley P, Lisanti MP, Dwek RA, Rudd PM, Moskovitz J, Epstein CJ, Cruz TD, Kuziel WA, Maeda N, Sap J, Ashe KH, Carlson GA, Tesseur I, Wyss-Coray T, Mucke L, Weisgraber KH, Mahley RW, Cohen FE, Prusiner SB. PMID: 18559949; PMCID: PMC2828448.
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    111. Mechanisms of prion protein assembly into amyloid. Proc Natl Acad Sci U S A. 2008 Feb 19; 105(7):2409-14. Stöhr J, Weinmann N, Wille H, Kaimann T, Nagel-Steger L, Birkmann E, Panza G, Prusiner SB, Eigen M, Riesner D. PMID: 18268326; PMCID: PMC2268150.
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    112. Small-molecule aggregates inhibit amyloid polymerization. Nat Chem Biol. 2008 Mar; 4(3):197-9. Feng BY, Toyama BH, Wille H, Colby DW, Collins SR, May BC, Prusiner SB, Weissman J, Shoichet BK. PMID: 18223646; PMCID: PMC2730835.
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    113. Prion detection by an amyloid seeding assay. Proc Natl Acad Sci U S A. 2007 Dec 26; 104(52):20914-9. Colby DW, Zhang Q, Wang S, Groth D, Legname G, Riesner D, Prusiner SB. PMID: 18096717; PMCID: PMC2409241.
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    114. Cell division modulates prion accumulation in cultured cells. Proc Natl Acad Sci U S A. 2007 Nov 13; 104(46):17971-6. Ghaemmaghami S, Phuan PW, Perkins B, Ullman J, May BC, Cohen FE, Prusiner SB. PMID: 17989223; PMCID: PMC2084281.
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    115. Influence of water, fat, and glycerol on the mechanism of thermal prion inactivation. J Biol Chem. 2007 Dec 07; 282(49):35855-67. Müller H, Stitz L, Wille H, Prusiner SB, Riesner D. PMID: 17878157.
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    116. Electron crystallography of the scrapie prion protein complexed with heavy metals. Arch Biochem Biophys. 2007 Nov 15; 467(2):239-48. Wille H, Govaerts C, Borovinskiy A, Latawiec D, Downing KH, Cohen FE, Prusiner SB. PMID: 17935686; PMCID: PMC3557509.
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    117. Polio and Nobel prizes: looking back 50 years. Ann Neurol. 2007 May; 61(5):385-95. Norrby E, Prusiner SB. PMID: 17469121.
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    118. Discriminating between cellular and misfolded prion protein by using affinity to 9-aminoacridine compounds. J Gen Virol. 2007 Apr; 88(Pt 4):1392-1401. Phuan PW, Zorn JA, Safar J, Giles K, Prusiner SB, Cohen FE, May BCH. PMID: 17374787.
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    119. Transgenic mouse brains for the evaluation and quality control of BSE tests. Biol Chem. 2007 Mar; 388(3):349-54. Philipp WJ, Groth D, Giles K, Vodrazka P, Schimmel H, Feyssaguet M, Toomik R, Schacher P, Osman AA, Lachmann I, Wear A, Arsac JN, Prusiner SB. PMID: 17338644.
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    120. Phosphorothioate oligonucleotides reduce PrP levels and prion infectivity in cultured cells. Mol Med. 2007 Mar-Apr; 13(3-4):190-8. Karpuj MV, Giles K, Gelibter-Niv S, Scott MR, Lingappa VR, Szoka FC, Peretz D, Denetclaw W, Prusiner SB. PMID: 17592554; PMCID: PMC1892763.
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    121. Thioaptamer interactions with prion proteins: sequence-specific and non-specific binding sites. J Mol Biol. 2007 Jun 15; 369(4):1001-14. King DJ, Safar JG, Legname G, Prusiner SB. PMID: 17481659.
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    122. Structure-activity relationship study of prion inhibition by 2-aminopyridine-3,5-dicarbonitrile-based compounds: parallel synthesis, bioactivity, and in vitro pharmacokinetics. J Med Chem. 2007 Jan 11; 50(1):65-73. May BC, Zorn JA, Witkop J, Sherrill J, Wallace AC, Legname G, Prusiner SB, Cohen FE. PMID: 17201410.
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    123. Developmental expression of PrP in the post-implantation embryo. Brain Res. 2007 Mar 30; 1139:60-7. Tremblay P, Bouzamondo-Bernstein E, Heinrich C, Prusiner SB, DeArmond SJ. PMID: 17292334; PMCID: PMC2706582.
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    124. Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proc Natl Acad Sci U S A. 2006 Dec 12; 103(50):19105-10. Legname G, Nguyen HO, Peretz D, Cohen FE, DeArmond SJ, Prusiner SB. PMID: 17142317; PMCID: PMC1748184.
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    125. Transmission of elk and deer prions to transgenic mice. J Virol. 2006 Sep; 80(18):9104-14. Tamgüney G, Giles K, Bouzamondo-Bernstein E, Bosque PJ, Miller MW, Safar J, DeArmond SJ, Prusiner SB. PMID: 16940522; PMCID: PMC1563923.
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    126. Expression mapping of tetracycline-responsive prion protein promoter: digital atlasing for generating cell-specific disease models. Neuroimage. 2006 Nov 01; 33(2):449-62. Boy J, Leergaard TB, Schmidt T, Odeh F, Bichelmeier U, Nuber S, Holzmann C, Wree A, Prusiner SB, Bujard H, Riess O, Bjaalie JG. PMID: 16931059.
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    127. Structure-activity relationship study of 9-aminoacridine compounds in scrapie-infected neuroblastoma cells. Bioorg Med Chem Lett. 2006 Sep 15; 16(18):4913-6. May BC, Witkop J, Sherrill J, Anderson MO, Madrid PB, Zorn JA, Prusiner SB, Cohen FE, Guy RK. PMID: 16860557.
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    128. Directed evolution of an anti-prion protein scFv fragment to an affinity of 1 pM and its structural interpretation. J Mol Biol. 2006 Oct 13; 363(1):75-97. Luginbühl B, Kanyo Z, Jones RM, Fletterick RJ, Prusiner SB, Cohen FE, Williamson RA, Burton DR, Plückthun A. PMID: 16962610.
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    129. Human prions and plasma lipoproteins. Proc Natl Acad Sci U S A. 2006 Jul 25; 103(30):11312-7. Safar JG, Wille H, Geschwind MD, Deering C, Latawiec D, Serban A, King DJ, Legname G, Weisgraber KH, Mahley RW, Miller BL, Dearmond SJ, Prusiner SB. PMID: 16849426; PMCID: PMC1544083.
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    130. Copper induces conformational changes in the N-terminal part of cell-surface PrPC. Arch Virol. 2006 Nov; 151(11):2103-9. Leclerc E, Serban H, Prusiner SB, Burton DR, Williamson RA. PMID: 16791441.
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    131. Changes in neuropeptide expression in mice infected with prions. Neurobiol Aging. 2007 May; 28(5):748-65. Diez M, Groth D, DeArmond SJ, Prusiner SB, Hökfelt T. PMID: 16621165.
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    132. Quinacrine is mainly metabolized to mono-desethyl quinacrine by CYP3A4/5 and its brain accumulation is limited by P-glycoprotein. Drug Metab Dispos. 2006 Jul; 34(7):1136-44. Huang Y, Okochi H, May BC, Legname G, Prusiner SB, Benet LZ, Guglielmo BJ, Lin ET. PMID: 16581945.
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    133. Prion infection of mouse neurospheres. Proc Natl Acad Sci U S A. 2006 Mar 07; 103(10):3875-80. Giri RK, Young R, Pitstick R, DeArmond SJ, Prusiner SB, Carlson GA. PMID: 16495413; PMCID: PMC1533787.
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    134. Discovering DNA encodes heredity and prions are infectious proteins. Annu Rev Genet. 2006; 40:25-45. Prusiner SB, McCarty M. PMID: 17094736.
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    135. Inactivation of prions by acidic sodium dodecyl sulfate. J Virol. 2006 Jan; 80(1):322-31. Peretz D, Supattapone S, Giles K, Vergara J, Freyman Y, Lessard P, Safar JG, Glidden DV, McCulloch C, Nguyen HO, Scott M, Dearmond SJ, Prusiner SB. PMID: 16352557; PMCID: PMC1317507.
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    136. Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro. J Neurochem. 2005 Dec; 95(5):1373-86. Kanaani J, Prusiner SB, Diacovo J, Baekkeskov S, Legname G. PMID: 16313516.
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    137. Solid-state NMR structural studies of the fibril form of a mutant mouse prion peptide PrP89-143(P101L). Solid State Nucl Magn Reson. 2006 Feb; 29(1-3):183-90. Lim KH, Nguyen TN, Damo SM, Mazur T, Ball HL, Prusiner SB, Pines A, Wemmer DE. PMID: 16256316.
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    138. Selective precipitation of prions by polyoxometalate complexes. J Am Chem Soc. 2005 Oct 12; 127(40):13802-3. Lee IS, Long JR, Prusiner SB, Safar JG. PMID: 16201796.
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    139. Prion clearance in bigenic mice. J Gen Virol. 2005 Oct; 86(Pt 10):2913-2923. Safar JG, DeArmond SJ, Kociuba K, Deering C, Didorenko S, Bouzamondo-Bernstein E, Prusiner SB, Tremblay P. PMID: 16186247.
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    140. Search for a prion-specific nucleic acid. J Virol. 2005 Aug; 79(16):10796-806. Safar JG, Kellings K, Serban A, Groth D, Cleaver JE, Prusiner SB, Riesner D. PMID: 16051871; PMCID: PMC1182634.
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    141. Assembly of natural and recombinant prion protein into fibrils. Biol Chem. 2005 Jun; 386(6):569-80. Leffers KW, Wille H, Stöhr J, Junger E, Prusiner SB, Riesner D. PMID: 16006244.
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    142. Transmission barriers for bovine, ovine, and human prions in transgenic mice. J Virol. 2005 May; 79(9):5259-71. Scott MR, Peretz D, Nguyen HO, Dearmond SJ, Prusiner SB. PMID: 15827140; PMCID: PMC1082721.
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    143. Diagnosis of human prion disease. Proc Natl Acad Sci U S A. 2005 Mar 01; 102(9):3501-6. Safar JG, Geschwind MD, Deering C, Didorenko S, Sattavat M, Sanchez H, Serban A, Vey M, Baron H, Giles K, Miller BL, Dearmond SJ, Prusiner SB. PMID: 15741275; PMCID: PMC552933.
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    144. Strain-specified characteristics of mouse synthetic prions. Proc Natl Acad Sci U S A. 2005 Feb 08; 102(6):2168-73. Legname G, Nguyen HO, Baskakov IV, Cohen FE, Dearmond SJ, Prusiner SB. PMID: 15671162; PMCID: PMC548557.
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    145. Notch-1 activation and dendritic atrophy in prion disease. Proc Natl Acad Sci U S A. 2005 Jan 18; 102(3):886-91. Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ. PMID: 15640354; PMCID: PMC545568.
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    146. Oxidation of methionine residues in the prion protein by hydrogen peroxide. Arch Biochem Biophys. 2004 Dec 15; 432(2):188-95. Requena JR, Dimitrova MN, Legname G, Teijeira S, Prusiner SB, Levine RL. PMID: 15542057.
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    147. Pharmacokinetics of quinacrine in the treatment of prion disease. BMC Infect Dis. 2004 Nov 29; 4:53. Yung L, Huang Y, Lessard P, Legname G, Lin ET, Baldwin M, Prusiner SB, Ryou C, Guglielmo BJ. PMID: 15569390; PMCID: PMC535929.
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    148. Immunoglobulins in urine of hamsters with scrapie. J Biol Chem. 2004 Nov 19; 279(47):48817-20. Serban A, Legname G, Hansen K, Kovaleva N, Prusiner SB. PMID: 15310752.
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    149. The neurodegeneration sequence in prion diseases: evidence from functional, morphological and ultrastructural studies of the GABAergic system. J Neuropathol Exp Neurol. 2004 Aug; 63(8):882-99. Bouzamondo-Bernstein E, Hopkins SD, Spilman P, Uyehara-Lock J, Deering C, Safar J, Prusiner SB, Ralston HJ, DeArmond SJ. PMID: 15330342.
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    150. Synthetic mammalian prions. Science. 2004 Jul 30; 305(5684):673-6. Legname G, Baskakov IV, Nguyen HO, Riesner D, Cohen FE, DeArmond SJ, Prusiner SB. PMID: 15286374.
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    151. Detecting mad cow disease. Sci Am. 2004 Jul; 291(1):86-93. Prusiner SB. PMID: 15255592.
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    152. Evidence for assembly of prions with left-handed beta-helices into trimers. Proc Natl Acad Sci U S A. 2004 Jun 01; 101(22):8342-7. Govaerts C, Wille H, Prusiner SB, Cohen FE. PMID: 15155909; PMCID: PMC420396.
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    153. Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues. Nat Biotechnol. 2004 Jun; 22(6):724-31. Schmitt-Ulms G, Hansen K, Liu J, Cowdrey C, Yang J, DeArmond SJ, Cohen FE, Prusiner SB, Baldwin MA. PMID: 15146195.
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    154. Prions: so many fibers, so little infectivity. Trends Biochem Sci. 2004 Apr; 29(4):162-5. May BC, Govaerts C, Prusiner SB, Cohen FE. PMID: 15124628.
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    155. The peculiar nature of unfolding of the human prion protein. Protein Sci. 2004 Mar; 13(3):586-95. Baskakov IV, Legname G, Gryczynski Z, Prusiner SB. PMID: 14767078; PMCID: PMC2286731.
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    156. When sporadic disease is not sporadic: the potential for genetic etiology. Arch Neurol. 2004 Feb; 61(2):213-6. Goldman JS, Miller BL, Safar J, de Tourreil S, Martindale JL, Prusiner SB, Geschwind MD. PMID: 14967768.
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    157. Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol. 2004 Feb; 78(4):2088-99. Tremblay P, Ball HL, Kaneko K, Groth D, Hegde RS, Cohen FE, DeArmond SJ, Prusiner SB, Safar JG. PMID: 14747574; PMCID: PMC369494.
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    158. Early evidence that a protease-resistant protein is an active component of the infectious prion. Cell. 2004 Jan 23; 116(2 Suppl):S109, 1 p following S113. Prusiner SB. PMID: 15055596.
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    159. Trafficking of prion proteins through a caveolae-mediated endosomal pathway. J Cell Biol. 2003 Aug 18; 162(4):703-17. Peters PJ, Mironov A, Peretz D, van Donselaar E, Leclerc E, Erpel S, DeArmond SJ, Burton DR, Williamson RA, Vey M, Prusiner SB. PMID: 12925711; PMCID: PMC2173792.
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    160. Cytosolic prion protein in neurons. J Neurosci. 2003 Aug 06; 23(18):7183-93. Mironov A, Latawiec D, Wille H, Bouzamondo-Bernstein E, Legname G, Williamson RA, Burton D, DeArmond SJ, Prusiner SB, Peters PJ. PMID: 12904479; PMCID: PMC6740654.
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    161. Copper coordination in the full-length, recombinant prion protein. Biochemistry. 2003 Jun 10; 42(22):6794-803. Burns CS, Aronoff-Spencer E, Legname G, Prusiner SB, Antholine WE, Gerfen GJ, Peisach J, Millhauser GL. PMID: 12779334; PMCID: PMC2905145.
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    162. Differential inhibition of prion propagation by enantiomers of quinacrine. Lab Invest. 2003 Jun; 83(6):837-43. Ryou C, Legname G, Peretz D, Craig JC, Baldwin MA, Prusiner SB. PMID: 12808118.
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    163. Cooperative binding of dominant-negative prion protein to kringle domains. J Mol Biol. 2003 May 30; 329(2):323-33. Ryou C, Prusiner SB, Legname G. PMID: 12758079.
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    164. Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A. 2003 Apr 15; 100(8):4784-9. Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J, Parchi P, Gambetti P, Will R, Ironside J, Heinrich C, Tremblay P, DeArmond SJ, Prusiner SB. PMID: 12684540; PMCID: PMC153633.
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    165. Potent inhibition of scrapie prion replication in cultured cells by bis-acridines. Proc Natl Acad Sci U S A. 2003 Mar 18; 100(6):3416-21. May BC, Fafarman AT, Hong SB, Rogers M, Deady LW, Prusiner SB, Cohen FE. PMID: 12626750; PMCID: PMC152307.
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    166. Perspectives on prion biology, prion disease pathogenesis, and pharmacologic approaches to treatment. Clin Lab Med. 2003 Mar; 23(1):1-41. DeArmond SJ, Prusiner SB. PMID: 12733423.
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    167. Conformation of PrP(C) on the cell surface as probed by antibodies. J Mol Biol. 2003 Feb 14; 326(2):475-83. Leclerc E, Peretz D, Ball H, Solforosi L, Legname G, Safar J, Serban A, Prusiner SB, Burton DR, Williamson RA. PMID: 12559915.
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    168. The PrP-like protein Doppel binds copper. J Biol Chem. 2003 Mar 14; 278(11):8888-96. Qin K, Coomaraswamy J, Mastrangelo P, Yang Y, Lugowski S, Petromilli C, Prusiner SB, Fraser PE, Goldberg JM, Chakrabartty A, Westaway D. PMID: 12482851.
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    169. A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron. 2002 Jun 13; 34(6):921-32. Peretz D, Williamson RA, Legname G, Matsunaga Y, Vergara J, Burton DR, DeArmond SJ, Prusiner SB, Scott MR. PMID: 12086640.
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    170. Pathway complexity of prion protein assembly into amyloid. J Biol Chem. 2002 Jun 14; 277(24):21140-8. Baskakov IV, Legname G, Baldwin MA, Prusiner SB, Cohen FE. PMID: 11912192.
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    171. Prions in skeletal muscle. Proc Natl Acad Sci U S A. 2002 Mar 19; 99(6):3812-7. Bosque PJ, Ryou C, Telling G, Peretz D, Legname G, DeArmond SJ, Prusiner SB. PMID: 11904434; PMCID: PMC122606.
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    172. Structural studies of the scrapie prion protein by electron crystallography. Proc Natl Acad Sci U S A. 2002 Mar 19; 99(6):3563-8. Wille H, Michelitsch MD, Guenebaut V, Supattapone S, Serban A, Cohen FE, Agard DA, Prusiner SB. PMID: 11891310; PMCID: PMC122563.
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    173. Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formation. J Mol Biol. 2002 Feb 22; 316(3):807-15. Nicholson EM, Mo H, Prusiner SB, Cohen FE, Marqusee S. PMID: 11866533.
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    174. Inherited prion disease caused by the V210I mutation: transmission to transgenic mice. Neurology. 2001 Dec 26; 57(12):2198-205. Mastrianni JA, Capellari S, Telling GC, Han D, Bosque P, Prusiner SB, DeArmond SJ. PMID: 11756597.
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    175. Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein. J Mol Biol. 2001 Dec 14; 314(5):1209-25. Schmitt-Ulms G, Legname G, Baldwin MA, Ball HL, Bradon N, Bosque PJ, Crossin KL, Edelman GM, DeArmond SJ, Cohen FE, Prusiner SB. PMID: 11743735.
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    176. Doppel-induced cerebellar degeneration in transgenic mice. Proc Natl Acad Sci U S A. 2001 Dec 18; 98(26):15288-93. Moore RC, Mastrangelo P, Bouzamondo E, Heinrich C, Legname G, Prusiner SB, Hood L, Westaway D, DeArmond SJ, Tremblay P. PMID: 11734625; PMCID: PMC65022.
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    177. Engineering the prion protein using chemical synthesis. J Pept Res. 2001 Nov; 58(5):357-74. Ball HL, King DS, Cohen FE, Prusiner SB, Baldwin MA. PMID: 11892845.
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    178. Induction of distinct [URE3] yeast prion strains. Mol Cell Biol. 2001 Oct; 21(20):7035-46. Schlumpberger M, Prusiner SB, Herskowitz I. PMID: 11564886; PMCID: PMC99879.
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    179. Solid-state NMR studies of the secondary structure of a mutant prion protein fragment of 55 residues that induces neurodegeneration. Proc Natl Acad Sci U S A. 2001 Sep 25; 98(20):11686-90. Laws DD, Bitter HM, Liu K, Ball HL, Kaneko K, Wille H, Cohen FE, Prusiner SB, Pines A, Wemmer DE. PMID: 11562491; PMCID: PMC58790.
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    180. Shattuck lecture--neurodegenerative diseases and prions. N Engl J Med. 2001 May 17; 344(20):1516-26. Prusiner SB. PMID: 11357156.
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    181. Folding of prion protein to its native alpha-helical conformation is under kinetic control. J Biol Chem. 2001 Jun 08; 276(23):19687-90. Baskakov IV, Legname G, Prusiner SB, Cohen FE. PMID: 11306559.
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    182. Prion glycoprotein: structure, dynamics, and roles for the sugars. Biochemistry. 2001 Apr 03; 40(13):3759-66. Rudd PM, Wormald MR, Wing DR, Prusiner SB, Dwek RA. PMID: 11300755.
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    183. Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form. EMBO J. 2001 Apr 02; 20(7):1547-54. Leclerc E, Peretz D, Ball H, Sakurai H, Legname G, Serban A, Prusiner SB, Burton DR, Williamson RA. PMID: 11285219; PMCID: PMC145482.
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    184. Conformational propagation with prion-like characteristics in a simple model of protein folding. Protein Sci. 2001 Apr; 10(4):819-35. Harrison PM, Chan HS, Prusiner SB, Cohen FE. PMID: 11274473; PMCID: PMC2373973.
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    185. Strain-specified relative conformational stability of the scrapie prion protein. Protein Sci. 2001 Apr; 10(4):854-63. Peretz D, Scott MR, Groth D, Williamson RA, Burton DR, Cohen FE, Prusiner SB. PMID: 11274476; PMCID: PMC2373967.
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    186. A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice. Mol Cell Biol. 2001 Apr; 21(7):2608-16. Supattapone S, Bouzamondo E, Ball HL, Wille H, Nguyen HO, Cohen FE, DeArmond SJ, Prusiner SB, Scott M. PMID: 11259607; PMCID: PMC86891.
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    187. Branched polyamines cure prion-infected neuroblastoma cells. J Virol. 2001 Apr; 75(7):3453-61. Supattapone S, Wille H, Uyechi L, Safar J, Tremblay P, Szoka FC, Cohen FE, Prusiner SB, Scott MR. PMID: 11238871; PMCID: PMC114138.
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    188. Local structural plasticity of the prion protein. Analysis of NMR relaxation dynamics. Biochemistry. 2001 Mar 06; 40(9):2743-53. Viles JH, Donne D, Kroon G, Prusiner SB, Cohen FE, Dyson HJ, Wright PE. PMID: 11258885.
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    189. Two different neurodegenerative diseases caused by proteins with similar structures. Proc Natl Acad Sci U S A. 2001 Feb 27; 98(5):2352-7. Mo H, Moore RC, Cohen FE, Westaway D, Prusiner SB, Wright PE, Dyson HJ. PMID: 11226243; PMCID: PMC30142.
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    190. Identification of two prion protein regions that modify scrapie incubation time. J Virol. 2001 Feb; 75(3):1408-13. Supattapone S, Muramoto T, Legname G, Mehlhorn I, Cohen FE, DeArmond SJ, Prusiner SB, Scott MR. PMID: 11152514; PMCID: PMC114047.
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    191. Affinity-tagged miniprion derivatives spontaneously adopt protease-resistant conformations. J Virol. 2000 Dec; 74(24):11928-34. Supattapone S, Nguyen HO, Muramoto T, Cohen FE, DeArmond SJ, Prusiner SB, Scott M. PMID: 11090193; PMCID: PMC112476.
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    192. Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy. Biochemistry. 2000 Nov 14; 39(45):13760-71. Aronoff-Spencer E, Burns CS, Avdievich NI, Gerfen GJ, Peisach J, Antholine WE, Ball HL, Cohen FE, Prusiner SB, Millhauser GL. PMID: 11076515; PMCID: PMC2903970.
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    193. Expression of unglycosylated mutated prion protein facilitates PrP(Sc) formation in neuroblastoma cells infected with different prion strains. J Gen Virol. 2000 Oct; 81(Pt 10):2555-2563. Korth C, Kaneko K, Prusiner SB. PMID: 10993946.
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    194. Quantitative trait loci affecting prion incubation time in mice. Genomics. 2000 Oct 01; 69(1):47-53. Stephenson DA, Chiotti K, Ebeling C, Groth D, DeArmond SJ, Prusiner SB, Carlson GA. PMID: 11013074.
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    195. Doppel is an N-glycosylated, glycosylphosphatidylinositol-anchored protein. Expression in testis and ectopic production in the brains of Prnp(0/0) mice predisposed to Purkinje cell loss. J Biol Chem. 2000 Sep 01; 275(35):26834-41. Silverman GL, Qin K, Moore RC, Yang Y, Mastrangelo P, Tremblay P, Prusiner SB, Cohen FE, Westaway D. PMID: 10842180.
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    196. Selective neuronal vulnerability during experimental scrapie infection: insights from an ultrastructural investigation. Brain Res. 2000 Aug 25; 874(2):210-5. Bouzamondo E, Milroy AM, Ralston HJ, Prusiner SB, DeArmond SJ. PMID: 10960606.
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    197. Structural changes in a hydrophobic domain of the prion protein induced by hydration and by ala-->Val and pro-->Leu substitutions. J Mol Biol. 2000 Jul 28; 300(5):1283-96. Inouye H, Bond J, Baldwin MA, Ball HL, Prusiner SB, Kirschner DA. PMID: 10903869.
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    198. Scrapie infectivity is independent of amyloid staining properties of the N-terminally truncated prion protein. J Struct Biol. 2000 Jun; 130(2-3):323-38. Wille H, Prusiner SB, Cohen FE. PMID: 10940236.
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    199. Mimicking dominant negative inhibition of prion replication through structure-based drug design. Proc Natl Acad Sci U S A. 2000 May 23; 97(11):6073-8. Perrier V, Wallace AC, Kaneko K, Safar J, Prusiner SB, Cohen FE. PMID: 10823951; PMCID: PMC18560.
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    200. Cultured cell sublines highly susceptible to prion infection. J Virol. 2000 May; 74(9):4377-86. Bosque PJ, Prusiner SB. PMID: 10756052; PMCID: PMC111954.
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    201. Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein. J Virol. 2000 May; 74(9):4351-60. Zulianello L, Kaneko K, Scott M, Erpel S, Han D, Cohen FE, Prusiner SB. PMID: 10756050; PMCID: PMC111952.
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    202. Self-assembly of recombinant prion protein of 106 residues. Biochemistry. 2000 Mar 14; 39(10):2792-804. Baskakov IV, Aagaard C, Mehlhorn I, Wille H, Groth D, Baldwin MA, Prusiner SB, Cohen FE. PMID: 10704232.
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    203. The prion domain of yeast Ure2p induces autocatalytic formation of amyloid fibers by a recombinant fusion protein. Protein Sci. 2000 Mar; 9(3):440-51. Schlumpberger M, Wille H, Baldwin MA, Butler DA, Herskowitz I, Prusiner SB. PMID: 10752606; PMCID: PMC2144574.
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    204. Copper binding to octarepeat peptides of the prion protein monitored by mass spectrometry. Protein Sci. 2000 Feb; 9(2):332-43. Whittal RM, Ball HL, Cohen FE, Burlingame AL, Prusiner SB, Baldwin MA. PMID: 10716185; PMCID: PMC2144551.
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    205. A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice. J Mol Biol. 2000 Jan 28; 295(4):997-1007. Kaneko K, Ball HL, Wille H, Zhang H, Groth D, Torchia M, Tremblay P, Safar J, Prusiner SB, DeArmond SJ, Baldwin MA, Cohen FE. PMID: 10656806.
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    206. Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A. 1999 Dec 21; 96(26):15137-42. Scott MR, Will R, Ironside J, Nguyen HO, Tremblay P, DeArmond SJ, Prusiner SB. PMID: 10611351; PMCID: PMC24786.
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    207. Transmissible and genetic prion diseases share a common pathway of neurodegeneration. Nature. 1999 Dec 16; 402(6763):822-6. Hegde RS, Tremblay P, Groth D, DeArmond SJ, Prusiner SB, Lingappa VR. PMID: 10617204.
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    208. Elimination of prions by branched polyamines and implications for therapeutics. Proc Natl Acad Sci U S A. 1999 Dec 07; 96(25):14529-34. Supattapone S, Nguyen HO, Cohen FE, Prusiner SB, Scott MR. PMID: 10588739; PMCID: PMC24470.
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    209. Kinetics of prion protein accumulation in the CNS of mice with experimental scrapie. J Neuropathol Exp Neurol. 1999 Dec; 58(12):1244-9. Tatzelt J, Groth DF, Torchia M, Prusiner SB, DeArmond SJ. PMID: 10604749.
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    210. Glycosylation differences between the normal and pathogenic prion protein isoforms. Proc Natl Acad Sci U S A. 1999 Nov 09; 96(23):13044-9. Rudd PM, Endo T, Colominas C, Groth D, Wheeler SF, Harvey DJ, Wormald MR, Serban H, Prusiner SB, Kobata A, Dwek RA. PMID: 10557270; PMCID: PMC23897.
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    211. Antibody binding defines a structure for an epitope that participates in the PrPC-->PrPSc conformational change. J Mol Biol. 1999 Nov 05; 293(4):855-63. Kanyo ZF, Pan KM, Williamson RA, Burton DR, Prusiner SB, Fletterick RJ, Cohen FE. PMID: 10543972.
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    212. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. J Mol Biol. 1999 Oct 01; 292(4):797-817. Moore RC, Lee IY, Silverman GL, Harrison PM, Strome R, Heinrich C, Karunaratne A, Pasternak SH, Chishti MA, Liang Y, Mastrangelo P, Wang K, Smit AF, Katamine S, Carlson GA, Cohen FE, Prusiner SB, Melton DW, Tremblay P, Hood LE, Westaway D. PMID: 10525406.
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    213. A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest. 1999 Jun; 79(6):689-97. Nishida N, Tremblay P, Sugimoto T, Shigematsu K, Shirabe S, Petromilli C, Erpel SP, Nakaoke R, Atarashi R, Houtani T, Torchia M, Sakaguchi S, DeArmond SJ, Prusiner SB, Katamine S. PMID: 10378511.
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    214. Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med. 1999 May 27; 340(21):1630-8. Mastrianni JA, Nixon R, Layzer R, Telling GC, Han D, DeArmond SJ, Prusiner SB. PMID: 10341275.
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    215. Solution structure of Syrian hamster prion protein rPrP(90-231). Biochemistry. 1999 Apr 27; 38(17):5362-77. Liu H, Farr-Jones S, Ulyanov NB, Llinas M, Marqusee S, Groth D, Cohen FE, Prusiner SB, James TL. PMID: 10220323.
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    216. Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice. Cell. 1999 Mar 19; 96(6):869-78. Supattapone S, Bosque P, Muramoto T, Wille H, Aagaard C, Peretz D, Nguyen HO, Heinrich C, Torchia M, Safar J, Cohen FE, DeArmond SJ, Prusiner SB, Scott M. PMID: 10102274.
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    217. Copper binding to the prion protein: structural implications of four identical cooperative binding sites. Proc Natl Acad Sci U S A. 1999 Mar 02; 96(5):2042-7. Viles JH, Cohen FE, Prusiner SB, Goodin DB, Wright PE, Dyson HJ. PMID: 10051591; PMCID: PMC26733.
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    218. Thermodynamics of model prions and its implications for the problem of prion protein folding. J Mol Biol. 1999 Feb 19; 286(2):593-606. Harrison PM, Chan HS, Prusiner SB, Cohen FE. PMID: 9973573.
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    219. Ultrastructural studies on scrapie prion protein crystals obtained from reverse micellar solutions. Biophys J. 1999 Feb; 76(2):1048-62. Wille H, Prusiner SB. PMID: 9916037; PMCID: PMC1300055.
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    220. Prions. Proc Natl Acad Sci U S A. 1998 Nov 10; 95(23):13363-83. Prusiner SB. PMID: 9811807; PMCID: PMC33918.
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    221. Rapid acquisition of beta-sheet structure in the prion protein prior to multimer formation. Biol Chem. 1998 Nov; 379(11):1307-17. Post K, Pitschke M, Schäfer O, Wille H, Appel TR, Kirsch D, Mehlhorn I, Serban H, Prusiner SB, Riesner D. PMID: 9865603.
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    222. Mapping the prion protein using recombinant antibodies. J Virol. 1998 Nov; 72(11):9413-8. Williamson RA, Peretz D, Pinilla C, Ball H, Bastidas RB, Rozenshteyn R, Houghten RA, Prusiner SB, Burton DR. PMID: 9765500; PMCID: PMC110372.
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    223. Doxycycline control of prion protein transgene expression modulates prion disease in mice. Proc Natl Acad Sci U S A. 1998 Oct 13; 95(21):12580-5. Tremblay P, Meiner Z, Galou M, Heinrich C, Petromilli C, Lisse T, Cayetano J, Torchia M, Mobley W, Bujard H, DeArmond SJ, Prusiner SB. PMID: 9770528; PMCID: PMC22873.
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    224. Complete genomic sequence and analysis of the prion protein gene region from three mammalian species. Genome Res. 1998 Oct; 8(10):1022-37. Lee IY, Westaway D, Smit AF, Wang K, Seto J, Chen L, Acharya C, Ankener M, Baskin D, Cooper C, Yao H, Prusiner SB, Hood LE. PMID: 9799790.
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    225. Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med. 1998 Oct; 4(10):1157-65. Safar J, Wille H, Itri V, Groth D, Serban H, Torchia M, Cohen FE, Prusiner SB. PMID: 9771749.
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    226. The three-dimensional structure of prion protein: implications for prion disease. Biochem Soc Trans. 1998 Aug; 26(3):481-6. Baldwin MA, James TL, Cohen FE, Prusiner SB. PMID: 9765900.
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    227. The prion diseases. Brain Pathol. 1998 Jul; 8(3):499-513. Prusiner SB. PMID: 9669700; PMCID: PMC8098303.
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    228. Prion protein selectively binds copper(II) ions. Biochemistry. 1998 May 19; 37(20):7185-93. Stöckel J, Safar J, Wallace AC, Cohen FE, Prusiner SB. PMID: 9585530.
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    229. Neuropeptide Y: some viewpoints on a multifaceted peptide in the normal and diseased nervous system. Brain Res Brain Res Rev. 1998 May; 26(2-3):154-66. Hökfelt T, Broberger C, Zhang X, Diez M, Kopp J, Xu Z, Landry M, Bao L, Schalling M, Koistinaho J, DeArmond SJ, Prusiner S, Gong J, Walsh JH. PMID: 9651513.
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    230. Prion protein biology. Cell. 1998 May 01; 93(3):337-48. Prusiner SB, Scott MR, DeArmond SJ, Cohen FE. PMID: 9590169.
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    231. A transmembrane form of the prion protein in neurodegenerative disease. Science. 1998 Feb 06; 279(5352):827-34. Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ, Prusiner SB, Lingappa VR. PMID: 9452375.
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    232. Changes in the localization of brain prion proteins during scrapie infection. Neurology. 1998 Jan; 50(1):1271-80; discussion 2. DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB. PMID: 9443448.
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    233. Pathologic conformations of prion proteins. Annu Rev Biochem. 1998; 67:793-819. Cohen FE, Prusiner SB. PMID: 9759504.
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    234. Molecular studies of prion diseases. Prog Brain Res. 1998; 117:421-34. Safar J, Prusiner SB. PMID: 9932423.
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    235. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad Sci U S A. 1997 Dec 23; 94(26):14279-84. Scott MR, Safar J, Telling G, Nguyen O, Groth D, Torchia M, Koehler R, Tremblay P, Walther D, Cohen FE, DeArmond SJ, Prusiner SB. PMID: 9405603; PMCID: PMC24939.
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    236. Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. Proc Natl Acad Sci U S A. 1997 Dec 09; 94(25):13452-7. Donne DG, Viles JH, Groth D, Mehlhorn I, James TL, Cohen FE, Prusiner SB, Wright PE, Dyson HJ. PMID: 9391046; PMCID: PMC28326.
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    237. Propagation of prion strains through specific conformers of the prion protein. J Virol. 1997 Dec; 71(12):9032-44. Scott MR, Groth D, Tatzelt J, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB. PMID: 9371560; PMCID: PMC230204.
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    238. Selective neuronal targeting in prion disease. Neuron. 1997 Dec; 19(6):1337-48. DeArmond SJ, Sánchez H, Yehiely F, Qiu Y, Ninchak-Casey A, Daggett V, Camerino AP, Cayetano J, Rogers M, Groth D, Torchia M, Tremblay P, Scott MR, Cohen FE, Prusiner SB. PMID: 9427256.
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    239. Prion protein expression in Chinese hamster ovary cells using a glutamine synthetase selection and amplification system. Protein Eng. 1997 Dec; 10(12):1465-73. Blochberger TC, Cooper C, Peretz D, Tatzelt J, Griffith OH, Baldwin MA, Prusiner SB. PMID: 9543009.
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    240. Marked decrease of neuropeptide Y Y2 receptor binding sites in the hippocampus in murine prion disease. Proc Natl Acad Sci U S A. 1997 Nov 25; 94(24):13267-72. Diez M, Koistinaho J, Dearmond SJ, Groth D, Prusiner SB, Hökfelt T. PMID: 9371835; PMCID: PMC24298.
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    241. A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis. J Virol. 1997 Nov; 71(11):8821-31. Schätzl HM, Laszlo L, Holtzman DM, Tatzelt J, DeArmond SJ, Weiner RI, Mobley WC, Prusiner SB. PMID: 9343242; PMCID: PMC192348.
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    242. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol. 1997 Oct 31; 273(3):614-22. Peretz D, Williamson RA, Matsunaga Y, Serban H, Pinilla C, Bastidas RB, Rozenshteyn R, James TL, Houghten RA, Cohen FE, Prusiner SB, Burton DR. PMID: 9356250.
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    243. Prion diseases and the BSE crisis. Science. 1997 Oct 10; 278(5336):245-51. Prusiner SB. PMID: 9323196.
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    244. Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc Natl Acad Sci U S A. 1997 Sep 16; 94(19):10069-74. Kaneko K, Zulianello L, Scott M, Cooper CM, Wallace AC, James TL, Cohen FE, Prusiner SB. PMID: 9294164; PMCID: PMC23307.
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    245. Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc Natl Acad Sci U S A. 1997 Sep 16; 94(19):10086-91. James TL, Liu H, Ulyanov NB, Farr-Jones S, Zhang H, Donne DG, Kaneko K, Groth D, Mehlhorn I, Prusiner SB, Cohen FE. PMID: 9294167; PMCID: PMC23313.
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    246. Molecular properties of complexes formed between the prion protein and synthetic peptides. J Mol Biol. 1997 Jul 25; 270(4):574-86. Kaneko K, Wille H, Mehlhorn I, Zhang H, Ball H, Cohen FE, Baldwin MA, Prusiner SB. PMID: 9245588.
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    247. Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nat Med. 1997 Jul; 3(7):750-5. Muramoto T, DeArmond SJ, Scott M, Telling GC, Cohen FE, Prusiner SB. PMID: 9212101.
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    248. Familial Creutzfeldt-Jakob disease. Codon 200 prion disease in Libyan Jews. Medicine (Baltimore). 1997 Jul; 76(4):227-37. Meiner Z, Gabizon R, Prusiner SB. PMID: 9279329.
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    249. Kinetics and mechanism of amyloid formation by the prion protein H1 peptide as determined by time-dependent ESR. Chem Biol. 1997 May; 4(5):345-55. Lundberg KM, Stenland CJ, Cohen FE, Prusiner SB, Millhauser GL. PMID: 9195875.
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    250. N-terminally tagged prion protein supports prion propagation in transgenic mice. Protein Sci. 1997 Apr; 6(4):825-33. Telling GC, Tremblay P, Torchia M, Dearmond SJ, Cohen FE, Prusiner SB. PMID: 9098892; PMCID: PMC2144756.
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    251. Physical studies of conformational plasticity in a recombinant prion protein. Biochemistry. 1997 Mar 25; 36(12):3543-53. Zhang H, Stockel J, Mehlhorn I, Groth D, Baldwin MA, Prusiner SB, James TL, Cohen FE. PMID: 9132005.
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    252. COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform. Proc Natl Acad Sci U S A. 1997 Mar 18; 94(6):2333-8. Kaneko K, Vey M, Scott M, Pilkuhn S, Cohen FE, Prusiner SB. PMID: 9122195; PMCID: PMC20088.
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    253. Failure to transmit disease from gray tremor mutant mice. J Virol. 1997 Mar; 71(3):2342-5. Carlson GA, Banks S, Lund D, Reichert C, Groth D, Torchia M, Dearmond SJ, Prusiner SB. PMID: 9032370; PMCID: PMC191343.
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    254. The prion folding problem. Curr Opin Struct Biol. 1997 Feb; 7(1):53-9. Harrison PM, Bamborough P, Daggett V, Prusiner SB, Cohen FE. PMID: 9032055.
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    255. Prion protein gene variation among primates. J Mol Biol. 1997 Jan 17; 265(2):257. Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB. PMID: 9020986.
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    256. Identification of candidate proteins binding to prion protein. Neurobiol Dis. 1997; 3(4):339-55. Yehiely F, Bamborough P, Da Costa M, Perry BJ, Thinakaran G, Cohen FE, Carlson GA, Prusiner SB. PMID: 9173930.
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    257. Genetics of prions. Annu Rev Genet. 1997; 31:139-75. Prusiner SB, Scott MR. PMID: 9442893.
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    258. Recombinant scrapie-like prion protein of 106 amino acids is soluble. Proc Natl Acad Sci U S A. 1996 Dec 24; 93(26):15457-62. Muramoto T, Scott M, Cohen FE, Prusiner SB. PMID: 8986833; PMCID: PMC26426.
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    259. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science. 1996 Dec 20; 274(5295):2079-82. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, Mastrianni J, Lugaresi E, Gambetti P, Prusiner SB. PMID: 8953038.
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    260. Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains. Proc Natl Acad Sci U S A. 1996 Dec 10; 93(25):14945-9. Vey M, Pilkuhn S, Wille H, Nixon R, DeArmond SJ, Smart EJ, Anderson RG, Taraboulos A, Prusiner SB. PMID: 8962161; PMCID: PMC26242.
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    261. Chemical chaperones interfere with the formation of scrapie prion protein. EMBO J. 1996 Dec 02; 15(23):6363-73. Tatzelt J, Prusiner SB, Welch WJ. PMID: 8978663; PMCID: PMC452460.
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    262. Molecular biology and pathogenesis of prion diseases. Trends Biochem Sci. 1996 Dec; 21(12):482-7. Prusiner SB. PMID: 9009832.
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    263. Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease. Neurology. 1996 Nov; 47(5):1305-12. Mastrianni JA, Iannicola C, Myers RM, DeArmond S, Prusiner SB. PMID: 8909447.
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    264. Prion biology and diseases--laughing cannibals, mad cows, and scientific heresy. Med Res Rev. 1996 Sep; 16(5):487-505. Prusiner SB. PMID: 8865151.
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    265. Decreased receptor-mediated calcium response in prion-infected cells correlates with decreased membrane fluidity and IP3 release. Neurology. 1996 Sep; 47(3):741-50. Wong K, Qiu Y, Hyun W, Nixon R, VanCleff J, Sanchez-Salazar J, Prusiner SB, DeArmond SJ. PMID: 8797473.
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    266. Aberrant induction of neuropeptide Y mRNA in hippocampal CA3 pyramidal neurones in scrapie-infected mice. Neuroreport. 1996 Aug 12; 7(12):1887-92. Diez M, Koistinaho J, DeArmond SJ, Camerino AP, Groth D, Caytano JC, Prusiner SB, Hökfelt T. PMID: 8905686.
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    267. Scrapie in mice deficient in apolipoprotein E or glial fibrillary acidic protein. Neurology. 1996 Aug; 47(2):449-53. Tatzelt J, Maeda N, Pekny M, Yang SL, Betsholtz C, Eliasson C, Cayetano J, Camerino AP, DeArmond SJ, Prusiner SB. PMID: 8757019.
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    268. Solid-state NMR studies of the prion protein H1 fragment. Protein Sci. 1996 Aug; 5(8):1655-61. Heller J, Kolbert AC, Larsen R, Ernst M, Bekker T, Baldwin M, Prusiner SB, Pines A, Wemmer DE. PMID: 8844854; PMCID: PMC2143492.
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    269. Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev. 1996 Jul 15; 10(14):1736-50. Telling GC, Haga T, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB. PMID: 8698234.
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    270. Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein. Proc Natl Acad Sci U S A. 1996 Jul 09; 93(14):7279-82. Williamson RA, Peretz D, Smorodinsky N, Bastidas R, Serban H, Mehlhorn I, DeArmond SJ, Prusiner SB, Burton DR. PMID: 8692983; PMCID: PMC38974.
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    273. Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc Natl Acad Sci U S A. 1996 Mar 19; 93(6):2403-7. Lledo PM, Tremblay P, DeArmond SJ, Prusiner SB, Nicoll RA. PMID: 8637886; PMCID: PMC39809.
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    274. Disruption of prion rods generates 10-nm spherical particles having high alpha-helical content and lacking scrapie infectivity. J Virol. 1996 Mar; 70(3):1714-22. Riesner D, Kellings K, Post K, Wille H, Serban H, Groth D, Baldwin MA, Prusiner SB. PMID: 8627692; PMCID: PMC189995.
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    276. Transgenetics and gene targeting in studies of prion diseases. Curr Top Microbiol Immunol. 1996; 207:95-123. Scott MR, Telling GC, Prusiner SB. PMID: 8575209.
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    278. Prion protein amyloid: separation of scrapie infectivity from PrP polymers. Ciba Found Symp. 1996; 199:181-99; discussion 199-201. Wille H, Baldwin MA, Cohen FE, DeArmond SJ, Prusiner SB. PMID: 8915611.
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    279. Lipid composition in scrapie-infected mouse brain: prion infection increases the levels of dolichyl phosphate and ubiquinone. J Neurochem. 1996 Jan; 66(1):277-85. Guan Z, Söderberg M, Sindelar P, Prusiner SB, Kristensson K, Dallner G. PMID: 8522965.
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    280. Structures of prion proteins and conformational models for prion diseases. Curr Top Microbiol Immunol. 1996; 207:49-67. Huang Z, Prusiner SB, Cohen FE. PMID: 8575206.
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    281. Human prion diseases and neurodegeneration. Curr Top Microbiol Immunol. 1996; 207:1-17. Prusiner SB. PMID: 8575200.
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    282. Transgenetics and neuropathology of prion diseases. Curr Top Microbiol Immunol. 1996; 207:125-46. DeArmond SJ, Prusiner SB. PMID: 8575201.
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    283. Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease. Nat Med. 1996 Jan; 2(1):59-64. Gabizon R, Telling G, Meiner Z, Halimi M, Kahana I, Prusiner SB. PMID: 8564843.
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    284. Molecular biology and genetics of prion diseases. Cold Spring Harb Symp Quant Biol. 1996; 61:473-93. Prusiner SB. PMID: 9246475.
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    285. Abnormal plasma membrane properties and functions in prion-infected cell lines. Cold Spring Harb Symp Quant Biol. 1996; 61:531-40. DeArmond SJ, Qiu Y, Wong K, Nixon R, Hyun W, Prusiner SB, Mobley WC. PMID: 9246479.
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    286. Prion protein structure and scrapie replication: theoretical, spectroscopic, and genetic investigations. Cold Spring Harb Symp Quant Biol. 1996; 61:495-509. Bamborough P, Wille H, Telling GC, Yehiely F, Prusiner SB, Cohen FE. PMID: 9246476.
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    287. Prion protein (PrP) synthetic peptides induce cellular PrP to acquire properties of the scrapie isoform. Proc Natl Acad Sci U S A. 1995 Nov 21; 92(24):11160-4. Kaneko K, Peretz D, Pan KM, Blochberger TC, Wille H, Gabizon R, Griffith OH, Cohen FE, Baldwin MA, Prusiner SB. PMID: 7479957; PMCID: PMC40591.
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    288. Prion disease (PrP-A117V) presenting with ataxia instead of dementia. Neurology. 1995 Nov; 45(11):2042-50. Mastrianni JA, Curtis MT, Oberholtzer JC, Da Costa MM, DeArmond S, Prusiner SB, Garbern JY. PMID: 7501157.
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    289. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell. 1995 Oct 06; 83(1):79-90. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, DeArmond SJ, Prusiner SB. PMID: 7553876.
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    290. Scanning for mutations in the human prion protein open reading frame by temporal temperature gradient gel electrophoresis. Electrophoresis. 1995 Oct; 16(10):1851-60. Wiese U, Wulfert M, Prusiner SB, Riesner D. PMID: 8586053.
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    291. X-ray diffraction of scrapie prion rods and PrP peptides. J Mol Biol. 1995 Sep 29; 252(4):412-22. Nguyen JT, Inouye H, Baldwin MA, Fletterick RJ, Cohen FE, Prusiner SB, Kirschner DA. PMID: 7563061.
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    292. Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene. Mol Med. 1995 Sep; 1(6):607-13. Spudich S, Mastrianni JA, Wrensch M, Gabizon R, Meiner Z, Kahana I, Rosenmann H, Kahana E, Prusiner SB. PMID: 8529127; PMCID: PMC2229975.
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    293. Prion protein isoforms, a convergence of biological and structural investigations. J Biol Chem. 1995 Aug 18; 270(33):19197-200. Baldwin MA, Cohen FE, Prusiner SB. PMID: 7642588.
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    294. Conformational transitions in peptides containing two putative alpha-helices of the prion protein. J Mol Biol. 1995 Jul 21; 250(4):514-26. Zhang H, Kaneko K, Nguyen JT, Livshits TL, Baldwin MA, Cohen FE, James TL, Prusiner SB. PMID: 7542350.
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    295. Molecular genetics and biophysics of prions. Uirusu. 1995 Jun; 45(1):5-42. Prusiner SB. PMID: 7571451.
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    296. Expression and targeting of Syrian hamster prion protein induced by heat shock in transgenic Drosophila melanogaster. Mech Dev. 1995 Jun; 51(2-3):317-27. Raeber AJ, Muramoto T, Kornberg TB, Prusiner SB. PMID: 7547477.
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    297. Theoretical studies of sequence effects on the conformational properties of a fragment of the prion protein: implications for scrapie formation. Chem Biol. 1995 May; 2(5):305-15. Kazmirski SL, Alonso DO, Cohen FE, Prusiner SB, Daggett V. PMID: 9383432.
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    298. Prion protein peptides induce alpha-helix to beta-sheet conformational transitions. Biochemistry. 1995 Apr 04; 34(13):4186-92. Nguyen J, Baldwin MA, Cohen FE, Prusiner SB. PMID: 7703230.
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    299. Etiology and pathogenesis of prion diseases. Am J Pathol. 1995 Apr; 146(4):785-811. DeArmond SJ, Prusiner SB. PMID: 7717447; PMCID: PMC1869256.
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    300. On safari with PrP: prion diseases of animals. Trends Microbiol. 1995 Apr; 3(4):141-7. Westaway D, Carlson GA, Prusiner SB. PMID: 7613755.
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    301. Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform. J Cell Biol. 1995 Apr; 129(1):121-32. Taraboulos A, Scott M, Semenov A, Avrahami D, Laszlo L, Prusiner SB, Avraham D. PMID: 7698979; PMCID: PMC2120366.
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    302. Scrapie prions selectively modify the stress response in neuroblastoma cells. Proc Natl Acad Sci U S A. 1995 Mar 28; 92(7):2944-8. Tatzelt J, Zuo J, Voellmy R, Scott M, Hartl U, Prusiner SB, Welch WJ. PMID: 7708753; PMCID: PMC42335.
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    303. Prion protein gene variation among primates. J Mol Biol. 1995 Jan 27; 245(4):362-74. Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB. PMID: 7837269.
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    304. The prion diseases. Sci Am. 1995 Jan; 272(1):48-51, 54-7. Prusiner SB. PMID: 7824915.
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    305. Prion protein transgenes and the neuropathology in prion diseases. Brain Pathol. 1995 Jan; 5(1):77-89. DeArmond SJ, Prusiner SB. PMID: 7767493.
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    306. Scrapie infection of transgenic mice leads to network and intrinsic dysfunction of cortical and hippocampal neurones. Neurobiol Dis. 1994 Nov; 1(1-2):25-30. Jefferys JG, Empson RM, Whittington MA, Prusiner SB. PMID: 9216983.
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    307. Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A. 1994 Oct 11; 91(21):9936-40. Telling GC, Scott M, Hsiao KK, Foster D, Yang SL, Torchia M, Sidle KC, Collinge J, DeArmond SJ, Prusiner SB. PMID: 7937921; PMCID: PMC44932.
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    308. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc Natl Acad Sci U S A. 1994 Sep 13; 91(19):9126-30. Hsiao KK, Groth D, Scott M, Yang SL, Serban H, Rapp D, Foster D, Torchia M, Dearmond SJ, Prusiner SB. PMID: 7916462; PMCID: PMC44760.
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    309. Neurodegeneration in humans caused by prions. West J Med. 1994 Sep; 161(3):264-72. Prusiner SB. PMID: 7975565; PMCID: PMC1011408.
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    310. Proposed three-dimensional structure for the cellular prion protein. Proc Natl Acad Sci U S A. 1994 Jul 19; 91(15):7139-43. Huang Z, Gabriel JM, Baldwin MA, Fletterick RJ, Prusiner SB, Cohen FE. PMID: 7913747; PMCID: PMC44354.
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    311. Structure and polymorphism of the mouse prion protein gene. Proc Natl Acad Sci U S A. 1994 Jul 05; 91(14):6418-22. Westaway D, Cooper C, Turner S, Da Costa M, Carlson GA, Prusiner SB. PMID: 7912827; PMCID: PMC44213.
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    312. Determinants of carboxyl-terminal domain translocation during prion protein biogenesis. J Biol Chem. 1994 Jun 17; 269(24):16810-20. De Fea KA, Nakahara DH, Calayag MC, Yost CS, Mirels LF, Prusiner SB, Lingappa VR. PMID: 7911469.
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    313. Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Sci U S A. 1994 Jun 07; 91(12):5690-4. Carlson GA, Ebeling C, Yang SL, Telling G, Torchia M, Groth D, Westaway D, DeArmond SJ, Prusiner SB. PMID: 7911243; PMCID: PMC44062.
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    314. Inherited prion diseases. Proc Natl Acad Sci U S A. 1994 May 24; 91(11):4611-4. Prusiner SB. PMID: 8197105; PMCID: PMC43836.
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    315. Structural clues to prion replication. Science. 1994 Apr 22; 264(5158):530-1. Cohen FE, Pan KM, Huang Z, Baldwin M, Fletterick RJ, Prusiner SB. PMID: 7909169.
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    316. Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Genes Dev. 1994 Apr 15; 8(8):959-69. Westaway D, Zuliani V, Cooper CM, Da Costa M, Neuman S, Jenny AL, Detwiler L, Prusiner SB. PMID: 7926780.
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    317. Human prion diseases. Ann Neurol. 1994 Apr; 35(4):385-95. Prusiner SB, Hsiao KK. PMID: 8154865.
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    318. Genetics of prion diseases and prion diversity in mice. Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29; 343(1306):363-9. Carlson GA, DeArmond SJ, Torchia M, Westaway D, Prusiner SB. PMID: 7913753.
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    319. Molecular biology and genetics of prion diseases. Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29; 343(1306):447-63. Prusiner SB. PMID: 7913765.
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    320. Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease. Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29; 343(1306):385-90. Gabizon R, Rosenman H, Meiner Z, Kahana I, Kahana E, Shugart Y, Ott J, Prusiner SB. PMID: 7913755.
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    321. The neuropathological phenotype in transgenic mice expressing different prion protein constructs. Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29; 343(1306):415-23. DeArmond SJ, Yang SL, Cayetano-Canlas J, Groth D, Prusiner SB. PMID: 7913760.
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    322. Nucleic acids in prion preparations: unspecific background or essential component? Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29; 343(1306):425-30. Kellings K, Prusiner SB, Riesner D. PMID: 7913761.
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    323. Biosynthesis of the prion proteins in scrapie-infected cells in culture. Braz J Med Biol Res. 1994 Feb; 27(2):303-7. Taraboulos A, Scott M, Semenov A, Avrahami D, Prusiner SB. PMID: 8081243.
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    324. Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats. Neurosci Lett. 1994 Jan 31; 166(2):171-4. Gomi H, Ikeda T, Kunieda T, Itohara S, Prusiner SB, Yamanouchi K. PMID: 7909925.
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    325. Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell. 1994 Jan 14; 76(1):117-29. Westaway D, DeArmond SJ, Cayetano-Canlas J, Groth D, Foster D, Yang SL, Torchia M, Carlson GA, Prusiner SB. PMID: 8287472.
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    326. Biology and genetics of prion diseases. Annu Rev Microbiol. 1994; 48:655-86. Prusiner SB. PMID: 7826022.
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    327. Detecting prion protein gene mutations by denaturing gradient gel electrophoresis. Hum Mutat. 1994; 4(1):42-50. Fink JK, Peacock ML, Warren JT, Roses AD, Prusiner SB. PMID: 7951257.
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    328. Prion diseases and neurodegeneration. Annu Rev Neurosci. 1994; 17:311-39. Prusiner SB, DeArmond SJ. PMID: 8210178.
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    329. A national strategy for development of effective methods for the prevention and treatment of Alzheimer's disease and related neurodegenerative disorders. Neurobiol Aging. 1994; 15 Suppl 2:S29-32. Prusiner SB. PMID: 7700455.
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    330. Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc Natl Acad Sci U S A. 1993 Nov 15; 90(22):10608-12. Prusiner SB, Groth D, Serban A, Koehler R, Foster D, Torchia M, Burton D, Yang SL, DeArmond SJ. PMID: 7902565; PMCID: PMC47826.
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    331. The neurochemistry of prion diseases. J Neurochem. 1993 Nov; 61(5):1589-601. DeArmond SJ, Prusiner SB. PMID: 7901326.
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    332. Genetic and infectious prion diseases. Arch Neurol. 1993 Nov; 50(11):1129-53. Prusiner SB. PMID: 8105771.
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    333. Scrapie prions alter receptor-mediated calcium responses in cultured cells. Neurology. 1993 Nov; 43(11):2335-41. Kristensson K, Feuerstein B, Taraboulos A, Hyun WC, Prusiner SB, DeArmond SJ. PMID: 8232952.
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    334. Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD). Am J Hum Genet. 1993 Oct; 53(4):828-35. Gabizon R, Rosenmann H, Meiner Z, Kahana I, Kahana E, Shugart Y, Ott J, Prusiner SB. PMID: 8105682; PMCID: PMC1682379.
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    335. Transgenetics and cell biology of prion diseases: investigations of PrPSc synthesis and diversity. Br Med Bull. 1993 Oct; 49(4):873-912. Prusiner SB. PMID: 8137134.
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    336. Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor. Glycobiology. 1993 Aug; 3(4):319-29. Borchelt DR, Rogers M, Stahl N, Telling G, Prusiner SB. PMID: 7691278.
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    337. Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. Proc Natl Acad Sci U S A. 1993 Jul 15; 90(14):6449-53. DeArmond SJ, Yang SL, Lee A, Bowler R, Taraboulos A, Groth D, Prusiner SB. PMID: 8101989; PMCID: PMC46949.
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    338. Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell. 1993 Jun 04; 73(5):979-88. Scott M, Groth D, Foster D, Torchia M, Yang SL, DeArmond SJ, Prusiner SB. PMID: 8098995.
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    339. Biology of prion diseases. J Acquir Immune Defic Syndr (1988). 1993 Jun; 6(6):663-5. Prusiner SB. PMID: 8496798.
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    340. Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. Proc Natl Acad Sci U S A. 1993 Apr 15; 90(8):3182-6. Rogers M, Yehiely F, Scott M, Prusiner SB. PMID: 8475059; PMCID: PMC46263.
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    341. Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse. Genetics. 1993 Apr; 133(4):979-88. Carlson GA, Ebeling C, Torchia M, Westaway D, Prusiner SB. PMID: 8462855; PMCID: PMC1205414.
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    342. Attempts to restore scrapie prion infectivity after exposure to protein denaturants. Proc Natl Acad Sci U S A. 1993 Apr 01; 90(7):2793-7. Prusiner SB, Groth D, Serban A, Stahl N, Gabizon R. PMID: 8464892; PMCID: PMC46182.
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    343. Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry. 1993 Mar 02; 32(8):1991-2002. Stahl N, Baldwin MA, Teplow DB, Hood L, Gibson BW, Burlingame AL, Prusiner SB. PMID: 8448158.
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    344. Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy. J Infect Dis. 1993 Mar; 167(3):602-13. Prusiner SB, Füzi M, Scott M, Serban D, Serban H, Taraboulos A, Gabriel JM, Wells GA, Wilesmith JW, Bradley R, et al. PMID: 8440932.
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    345. Transgenetic investigations of prion diseases of humans and animals. Philos Trans R Soc Lond B Biol Sci. 1993 Feb 27; 339(1288):239-54. Prusiner SB. PMID: 8097053.
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    346. Analysis of nucleic acids in purified scrapie prion preparations. Arch Virol Suppl. 1993; 7:215-25. Kellings K, Meyer N, Mirenda C, Prusiner SB, Riesner D. PMID: 8219805.
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    347. Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity. Proc Natl Acad Sci U S A. 1993 Jan 01; 90(1):1-5. Gasset M, Baldwin MA, Fletterick RJ, Prusiner SB. PMID: 8419912; PMCID: PMC45587.
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    348. Prion encephalopathies of animals and humans. Dev Biol Stand. 1993; 80:31-44. Prusiner SB. PMID: 8270114.
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    349. Prions and nucleic acids: search for "residual" nucleic acids and screening for mutations in the PrP-gene. Dev Biol Stand. 1993; 80:173-81. Riesner D, Kellings K, Wiese U, Wulfert M, Mirenda C, Prusiner SB. PMID: 8270107.
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    350. Chemistry and biology of prions. Biochemistry. 1992 Dec 15; 31(49):12277-88. Prusiner SB. PMID: 1463717.
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    351. Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. Proc Natl Acad Sci U S A. 1992 Nov 15; 89(22):10940-4. Gasset M, Baldwin MA, Lloyd DH, Gabriel JM, Holtzman DM, Cohen F, Fletterick R, Prusiner SB. PMID: 1438300; PMCID: PMC50458.
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    352. Molecular cloning of a candidate chicken prion protein. Proc Natl Acad Sci U S A. 1992 Oct 01; 89(19):9097-101. Gabriel JM, Oesch B, Kretzschmar H, Scott M, Prusiner SB. PMID: 1409608; PMCID: PMC50072.
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    353. Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems. J Virol. 1992 Oct; 66(10):6155-63. Raeber AJ, Borchelt DR, Scott M, Prusiner SB. PMID: 1356161; PMCID: PMC283664.
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    354. Purification and properties of the cellular prion protein from Syrian hamster brain. Protein Sci. 1992 Oct; 1(10):1343-52. Pan KM, Stahl N, Prusiner SB. PMID: 1363897; PMCID: PMC2142104.
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    355. Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Proc Natl Acad Sci U S A. 1992 Oct 01; 89(19):9349-53. Giaccone G, Verga L, Bugiani O, Frangione B, Serban D, Prusiner SB, Farlow MR, Ghetti B, Tagliavini F. PMID: 1357663; PMCID: PMC50124.
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    356. Molecular cloning of a mink prion protein gene. J Gen Virol. 1992 Oct; 73 ( Pt 10):2757-61. Kretzschmar HA, Neumann M, Riethmüller G, Prusiner SB. PMID: 1383401.
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    357. Natural and experimental prion diseases of humans and animals. Curr Opin Neurobiol. 1992 Oct; 2(5):638-47. Prusiner SB. PMID: 1422120.
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    358. Regional mapping of prion proteins in brain. Proc Natl Acad Sci U S A. 1992 Aug 15; 89(16):7620-4. Taraboulos A, Jendroska K, Serban D, Yang SL, DeArmond SJ, Prusiner SB. PMID: 1354357; PMCID: PMC49762.
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    359. Evidence for synthesis of scrapie prion proteins in the endocytic pathway. J Biol Chem. 1992 Aug 15; 267(23):16188-99. Borchelt DR, Taraboulos A, Prusiner SB. PMID: 1353761.
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    360. Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci. 1992 Aug; 1(8):986-97. Scott MR, Köhler R, Foster D, Prusiner SB. PMID: 1338978; PMCID: PMC2142161.
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    361. Synthesis and trafficking of prion proteins in cultured cells. Mol Biol Cell. 1992 Aug; 3(8):851-63. Taraboulos A, Raeber AJ, Borchelt DR, Serban D, Prusiner SB. PMID: 1356522; PMCID: PMC275644.
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    362. Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes Dev. 1992 Jul; 6(7):1213-28. Hecker R, Taraboulos A, Scott M, Pan KM, Yang SL, Torchia M, Jendroska K, DeArmond SJ, Prusiner SB. PMID: 1628828.
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    363. Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob disease. Neurology. 1992 Jul; 42(7):1355-60. Meiner Z, Halimi M, Polakiewicz RD, Prusiner SB, Gabizon R. PMID: 1352391.
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    364. Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid. Biochemistry. 1992 Jun 02; 31(21):5043-53. Stahl N, Baldwin MA, Hecker R, Pan KM, Burlingame AL, Prusiner SB. PMID: 1350920.
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    365. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature. 1992 Apr 16; 356(6370):577-82. Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp HP, DeArmond SJ, Prusiner SB, Aguet M, Weissmann C. PMID: 1373228.
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    366. Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocusing gel electrophoresis. J Gen Virol. 1992 Apr; 73 ( Pt 4):1025-9. Kellings K, Meyer N, Mirenda C, Prusiner SB, Riesner D. PMID: 1353106.
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    367. Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene. Nat Genet. 1992 Apr; 1(1):64-7. Dlouhy SR, Hsiao K, Farlow MR, Foroud T, Conneally PM, Johnson P, Prusiner SB, Hodes ME, Ghetti B. PMID: 1363809.
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    368. Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Nat Genet. 1992 Apr; 1(1):68-71. Hsiao K, Dlouhy SR, Farlow MR, Cass C, Da Costa M, Conneally PM, Hodes ME, Ghetti B, Prusiner SB. PMID: 1363810.
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    369. Prion protein mutation at codon 102 in an Italian family with Gerstmann-Sträussler-Scheinker syndrome. Neurology. 1992 Apr; 42(4):809-10. Kretzschmar HA, Kufer P, Riethmüller G, DeArmond S, Prusiner SB, Schiffer D. PMID: 1348851.
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    370. Molecular biology and genetics of neurodegenerative diseases caused by prions. Adv Virus Res. 1992; 41:241-80. Prusiner SB. PMID: 1575084.
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    371. Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. Lab Invest. 1991 Dec; 65(6):622-30. McKinley MP, Taraboulos A, Kenaga L, Serban D, Stieber A, DeArmond SJ, Prusiner SB, Gonatas N. PMID: 1684401.
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    372. Epitope mapping of the Syrian hamster prion protein utilizing chimeric and mutant genes in a vaccinia virus expression system. J Immunol. 1991 Nov 15; 147(10):3568-74. Rogers M, Serban D, Gyuris T, Scott M, Torchia T, Prusiner SB. PMID: 1719082.
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    373. Prions and prion proteins. FASEB J. 1991 Oct; 5(13):2799-807. Stahl N, Prusiner SB. PMID: 1916104.
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    374. Electrospray mass spectrometry of the glycosylinositol phospholipid of the scrapie prion protein. Cell Biol Int Rep. 1991 Sep; 15(9):853-62. Stahl N, Baldwin MA, Prusiner SB. PMID: 1686992.
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    375. Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity. Neurology. 1991 Sep; 41(9):1482-90. Jendroska K, Heinzel FP, Torchia M, Stowring L, Kretzschmar HA, Kon A, Stern A, Prusiner SB, DeArmond SJ. PMID: 1679911.
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    376. Molecular biology and pathology of scrapie and the prion diseases of humans. Brain Pathol. 1991 Jul; 1(4):297-310. Prusiner SB, DeArmond SJ. PMID: 1669719.
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    377. Molecular biology of prion diseases. Science. 1991 Jun 14; 252(5012):1515-22. Prusiner SB. PMID: 1675487.
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    378. Infectious and genetic manifestations of prion diseases. Mol Plant Microbe Interact. 1991 May-Jun; 4(3):226-33. Prusiner SB, Westaway D. PMID: 1932814.
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    379. A prion protein variant in a family with the telencephalic form of Gerstmann-Sträussler-Scheinker syndrome. Neurology. 1991 May; 41(5):681-4. Hsiao KK, Cass C, Schellenberg GD, Bird T, Devine-Gage E, Wisniewski H, Prusiner SB. PMID: 1674116.
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    380. Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med. 1991 Apr 18; 324(16):1091-7. Hsiao K, Meiner Z, Kahana E, Cass C, Kahana I, Avrahami D, Scarlato G, Abramsky O, Prusiner SB, Gabizon R. PMID: 2008182.
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    381. Molecular biology and genetics of prions--implications for sheep scrapie, "mad cows" and the BSE epidemic. Historical background. Cornell Vet. 1991 Apr; 81(2):85-101. Prusiner SB, Torchia M, Westaway D. PMID: 2029844.
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    382. Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. EMBO J. 1991 Mar; 10(3):513-9. Tagliavini F, Prelli F, Ghiso J, Bugiani O, Serban D, Prusiner SB, Farlow MR, Ghetti B, Frangione B. PMID: 1672107; PMCID: PMC452678.
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    383. Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis. J Virol. 1991 Mar; 65(3):1340-51. McKinley MP, Meyer RK, Kenaga L, Rahbar F, Cotter R, Serban A, Prusiner SB. PMID: 1704926; PMCID: PMC239910.
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    384. Genetics of prion infections. Trends Genet. 1991 Feb; 7(2):61-5. Carlson GA, Hsiao K, Oesch B, Westaway D, Prusiner SB. PMID: 1903568.
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    385. Ultrastructural studies of prions. Curr Top Microbiol Immunol. 1991; 172:75-91. McKinley MP, Prusiner SB. PMID: 1725771.
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    386. Molecular genetics and transgenic model of Gertsmann-Sträussler-Scheinker disease. Alzheimer Dis Assoc Disord. 1991; 5(3):155-62. Hsiao K, Prusiner SB. PMID: 1685324.
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    387. Prion protein genes: evolutionary and functional aspects. Curr Top Microbiol Immunol. 1991; 172:109-24. Oesch B, Westaway D, Prusiner SB. PMID: 1687379.
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    388. Novel properties and biology of scrapie prions. Curr Top Microbiol Immunol. 1991; 172:233-57. Prusiner SB. PMID: 1687382.
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    389. Prion biology and diseases. Harvey Lect. 1991-1992; 87:85-114. Prusiner SB. PMID: 1670551.
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    390. Spontaneous neurodegeneration in transgenic mice with prion protein codon 101 proline----leucine substitution. Ann N Y Acad Sci. 1991; 640:166-70. Hsiao K, Scott M, Foster D, DeArmond SJ, Groth D, Serban H, Prusiner SB. PMID: 1685642.
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    391. Molecular biology and transgenetics of prion diseases. Crit Rev Biochem Mol Biol. 1991; 26(5-6):397-438. Prusiner SB. PMID: 1684745.
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    392. Molecular biology of prions causing infectious and genetic encephalopathies of humans as well as scrapie of sheep and BSE of cattle. Dev Biol Stand. 1991; 75:55-74. Prusiner SB. PMID: 1686599.
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    393. Search for a putative scrapie genome in purified prion fractions reveals a paucity of nucleic acids. J Gen Virol. 1991 Jan; 72 ( Pt 1):37-49. Meyer N, Rosenbaum V, Schmidt B, Gilles K, Mirenda C, Groth D, Prusiner SB, Riesner D. PMID: 1899270.
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    394. Acceleration of scrapie in trisomy 16----diploid aggregation chimeras. Ann Neurol. 1991 Jan; 29(1):95-7. Epstein CJ, Foster DB, DeArmond SJ, Prusiner SB. PMID: 1996884.
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    395. Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science. 1990 Dec 14; 250(4987):1587-90. Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB. PMID: 1980379.
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    396. Inherited human prion diseases. Neurology. 1990 Dec; 40(12):1820-7. Hsiao K, Prusiner SB. PMID: 2247227.
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    397. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell. 1990 Nov 16; 63(4):673-86. Prusiner SB, Scott M, Foster D, Pan KM, Groth D, Mirenda C, Torchia M, Yang SL, Serban D, Carlson GA, et al. PMID: 1977523.
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    398. Permethylation and tandem mass spectrometry of oligosaccharides having free hexosamine: analysis of the glycoinositol phospholipid anchor glycan from the scrapie prion protein. Anal Biochem. 1990 Nov 15; 191(1):174-82. Baldwin MA, Stahl N, Reinders LG, Gibson BW, Prusiner SB, Burlingame AL. PMID: 1981823.
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    399. Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie. Lab Invest. 1990 Nov; 63(5):601-11. Snow AD, Wight TN, Nochlin D, Koike Y, Kimata K, DeArmond SJ, Prusiner SB. PMID: 1977959.
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    400. Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation. Proc Natl Acad Sci U S A. 1990 Nov; 87(21):8262-6. Taraboulos A, Rogers M, Borchelt DR, McKinley MP, Scott M, Serban D, Prusiner SB. PMID: 1978322; PMCID: PMC54935.
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    401. Novel structure and genetics of prions causing neurodegeneration in humans and animals. Biologicals. 1990 Oct; 18(4):247-62. Prusiner SB. PMID: 1981006.
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    402. Identification of glycoinositol phospholipid linked and truncated forms of the scrapie prion protein. Biochemistry. 1990 Sep 25; 29(38):8879-84. Stahl N, Baldwin MA, Burlingame AL, Prusiner SB. PMID: 1980209.
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    403. Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked glycosylation sites. Glycobiology. 1990 Sep; 1(1):101-9. Rogers M, Taraboulos A, Scott M, Groth D, Prusiner SB. PMID: 1983782.
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    404. Link between scrapie and BSE? Nature. 1990 Jul 12; 346(6280):113. Westaway D, Prusiner SB. PMID: 2366871.
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    405. Identification of cellular proteins binding to the scrapie prion protein. Biochemistry. 1990 Jun 19; 29(24):5848-55. Oesch B, Teplow DB, Stahl N, Serban D, Hood LE, Prusiner SB. PMID: 1974464.
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    406. Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C. Biochemistry. 1990 Jun 05; 29(22):5405-12. Stahl N, Borchelt DR, Prusiner SB. PMID: 1974460.
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    407. Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells. J Cell Biol. 1990 Jun; 110(6):2117-32. Taraboulos A, Serban D, Prusiner SB. PMID: 1693623; PMCID: PMC2116143.
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    408. Unusual topogenic sequence directs prion protein biogenesis. Science. 1990 Apr 13; 248(4952):226-9. Lopez CD, Yost CS, Prusiner SB, Myers RM, Lingappa VR. PMID: 1970195.
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    409. Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins. Mol Cell Biol. 1990 Mar; 10(3):1153-63. Lowenstein DH, Butler DA, Westaway D, McKinley MP, DeArmond SJ, Prusiner SB. PMID: 2406562; PMCID: PMC360985.
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    410. DNA length polymorphism 5' to the myelin basic protein gene is associated with multiple sclerosis. Ann Neurol. 1990 Mar; 27(3):291-7. Boylan KB, Takahashi N, Paty DW, Sadovnick AD, Diamond M, Hood LE, Prusiner SB. PMID: 1691612.
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    411. Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. J Cell Biol. 1990 Mar; 110(3):743-52. Borchelt DR, Scott M, Taraboulos A, Stahl N, Prusiner SB. PMID: 1968466; PMCID: PMC2116048.
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    412. Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein. Nature. 1990 Feb 15; 343(6259):669-72. Yost CS, Lopez CD, Prusiner SB, Myers RM, Lingappa VR. PMID: 1968226.
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    413. Prion liposomes. Biochem J. 1990 Feb 15; 266(1):1-14. Gabizon R, Prusiner SB. PMID: 1968741; PMCID: PMC1131089.
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    414. Repetitive DNA (TGGA)n 5' to the human myelin basic protein gene: a new form of oligonucleotide repetitive sequence showing length polymorphism. Genomics. 1990 Jan; 6(1):16-22. Boylan KB, Ayres TM, Popko B, Takahashi N, Hood LE, Prusiner SB. PMID: 1689270.
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    415. Nerve growth factor induces gene expression of the prion protein and beta-amyloid protein precursor in the developing hamster central nervous system. Prog Brain Res. 1990; 86:227-38. McKinley MP, Longo FM, Valletta JS, Rahbar F, Neve RL, Prusiner SB, Mobley WC. PMID: 1982367.
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    416. Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins. Neurology. 1990 Jan; 40(1):110-7. Serban D, Taraboulos A, DeArmond SJ, Prusiner SB. PMID: 1967489.
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    417. Prion diseases of the central nervous system. Monogr Pathol. 1990; (32):86-122. Prusiner SB, DeArmond SJ. PMID: 2192281.
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    418. Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell. 1989 Dec 01; 59(5):847-57. Scott M, Foster D, Mirenda C, Serban D, Coufal F, Wälchli M, Torchia M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB. PMID: 2574076.
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    419. Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. Biochemistry. 1989 Oct 17; 28(21):8380-8. Endo T, Groth D, Prusiner SB, Kobata A. PMID: 2574992.
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    420. Primary structure of prion protein may modify scrapie isolate properties. Proc Natl Acad Sci U S A. 1989 Oct; 86(19):7475-9. Carlson GA, Westaway D, DeArmond SJ, Peterson-Torchia M, Prusiner SB. PMID: 2798418; PMCID: PMC298087.
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    421. Acceleration of scrapie in neonatal Syrian hamsters. Neurology. 1989 Oct; 39(10):1319-24. McKinley MP, DeArmond SJ, Torchia M, Mobley WC, Prusiner SB. PMID: 2507957.
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    422. Unraveling prion diseases through molecular genetics. Trends Neurosci. 1989 Jun; 12(6):221-7. Westaway D, Carlson GA, Prusiner SB. PMID: 2473558.
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    423. Creutzfeldt-Jakob disease and scrapie prions. Alzheimer Dis Assoc Disord. 1989 Spring-Summer; 3(1-2):52-78. Prusiner SB. PMID: 2568118.
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    424. Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature. 1989 Mar 23; 338(6213):342-5. Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB. PMID: 2564168.
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    425. Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet. 1989 Jan 07; 1(8628):51-2. Owen F, Poulter M, Lofthouse R, Collinge J, Crow TJ, Risby D, Baker HF, Ridley RM, Hsiao K, Prusiner SB. PMID: 2563037.
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    426. PrPSc in scrapie-infected hamster brain is spatially and temporally related to histopathology and infectivity titer. Prog Clin Biol Res. 1989; 317:601-18. De Armond SJ, Gonzales M, Mobley WC, Kon AA, Stern A, Prusiner H, Prusiner SB. PMID: 2574872.
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    427. Immunoaffinity purification and neutralization of scrapie prions. Prog Clin Biol Res. 1989; 317:583-600. Gabizon R, McKinley MP, Groth D, Westaway D, DeArmond SJ, Carlson GA, Prusiner SB. PMID: 2574871.
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    428. Sulfated glycosaminoglycans in amyloid plaques of prion diseases. Acta Neuropathol. 1989; 77(4):337-42. Snow AD, Kisilevsky R, Willmer J, Prusiner SB, DeArmond SJ. PMID: 2523631.
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    429. Scrapie prions. Annu Rev Microbiol. 1989; 43:345-74. Prusiner SB. PMID: 2572197.
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    430. Nerve growth factor increases mRNA levels for the prion protein and the beta-amyloid protein precursor in developing hamster brain. Proc Natl Acad Sci U S A. 1988 Dec; 85(24):9811-5. Mobley WC, Neve RL, Prusiner SB, McKinley MP. PMID: 2904679; PMCID: PMC282871.
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    431. Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time. Mol Cell Biol. 1988 Dec; 8(12):5528-40. Carlson GA, Goodman PA, Lovett M, Taylor BA, Marshall ST, Peterson-Torchia M, Westaway D, Prusiner SB. PMID: 3149717; PMCID: PMC365657.
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    432. CNS amyloid proteins in neurodegenerative diseases. Neurology. 1988 Oct; 38(10):1534-40. Roberts GW, Lofthouse R, Allsop D, Landon M, Kidd M, Prusiner SB, Crow TJ. PMID: 2901696.
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    433. Purification and properties of the cellular and scrapie hamster prion proteins. Eur J Biochem. 1988 Sep 01; 176(1):21-30. Turk E, Teplow DB, Hood LE, Prusiner SB. PMID: 3138115.
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    434. Immunoaffinity purification and neutralization of scrapie prion infectivity. Proc Natl Acad Sci U S A. 1988 Sep; 85(18):6617-21. Gabizon R, McKinley MP, Groth D, Prusiner SB. PMID: 3137571; PMCID: PMC282028.
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    435. Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da. Virology. 1988 Jun; 164(2):537-41. Bellinger-Kawahara CG, Kempner E, Groth D, Gabizon R, Prusiner SB. PMID: 3130718.
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    436. Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins. J Virol. 1988 May; 62(5):1558-64. Butler DA, Scott MR, Bockman JM, Borchelt DR, Taraboulos A, Hsiao KK, Kingsbury DT, Prusiner SB. PMID: 3282080; PMCID: PMC253182.
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    437. Properties of scrapie prion protein liposomes. J Biol Chem. 1988 Apr 05; 263(10):4950-5. Gabizon R, McKinley MP, Groth DF, Kenaga L, Prusiner SB. PMID: 3350818.
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    438. Prion protein gene expression in cultured cells. Protein Eng. 1988 Apr; 2(1):69-76. Scott MR, Butler DA, Bredesen DE, Wälchli M, Hsiao KK, Prusiner SB. PMID: 2908139.
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    439. Characterization of prion proteins with monospecific antisera to synthetic peptides. J Immunol. 1988 Feb 15; 140(4):1188-93. Barry RA, Vincent MT, Kent SB, Hood LE, Prusiner SB. PMID: 3125249.
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    440. Search for a scrapie-specific nucleic acid: a progress report. Ciba Found Symp. 1988; 135:209-23. Oesch B, Groth DF, Prusiner SB, Weissmann C. PMID: 3137001.
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    441. Novel mechanisms of degeneration of the central nervous system--prion structure and biology. Ciba Found Symp. 1988; 135:239-60. Prusiner SB, Stahl N, DeArmond SJ. PMID: 2900720.
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    442. Developmental regulation of prion protein mRNA in brain. Ciba Found Symp. 1988; 135:101-16. McKinley MP, Lingappa VR, Prusiner SB. PMID: 2900716.
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    443. Properties of scrapie prion proteins in liposomes and amyloid rods. Ciba Found Symp. 1988; 135:182-96. Gabizon R, McKinley MP, Prusiner SB. PMID: 2900719.
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    444. Genetic control of prion incubation period in mice. Ciba Found Symp. 1988; 135:84-99. Carlson GA, Westaway D, Goodman PA, Peterson M, Marshall ST, Prusiner SB. PMID: 2900721.
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    445. Molecular structure, biology, and genetics of prions. Adv Virus Res. 1988; 35:83-136. Prusiner SB. PMID: 2906782.
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    446. Prions and neurodegenerative diseases. N Engl J Med. 1987 Dec 17; 317(25):1571-81. Prusiner SB. PMID: 3317055.
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    447. Evidence for a secretory form of the cellular prion protein. Biochemistry. 1987 Dec 15; 26(25):8110-5. Hay B, Prusiner SB, Lingappa VR. PMID: 3126796.
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    448. Immuno-gold localization of prion filaments in scrapie-infected hamster brains. Lab Invest. 1987 Dec; 57(6):646-56. Wiley CA, Burrola PG, Buchmeier MJ, Wooddell MK, Barry RA, Prusiner SB, Lampert PW. PMID: 2891874.
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    449. The burden of proof in linking AIDS to scrapie. Nature. 1987 Dec 10-16; 330(6148):525-6. Braun MJ, Gonda MA, George DG, Bazan JF, Fletterick RJ, Prusiner SB. PMID: 2891035.
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    450. Distinct prion proteins in short and long scrapie incubation period mice. Cell. 1987 Nov 20; 51(4):651-62. Westaway D, Goodman PA, Mirenda CA, McKinley MP, Carlson GA, Prusiner SB. PMID: 2890436.
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    451. Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell. 1987 Oct 23; 51(2):229-40. Stahl N, Borchelt DR, Hsiao K, Prusiner SB. PMID: 2444340.
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    453. Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids. Lab Invest. 1987 Aug; 57(2):230-6. Kitamoto T, Ogomori K, Tateishi J, Prusiner SB. PMID: 2441141.
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    455. Scrapie infection diminishes spines and increases varicosities of dendrites in hamsters: a quantitative Golgi analysis. J Neuropathol Exp Neurol. 1987 Jul; 46(4):461-73. Hogan RN, Baringer JR, Prusiner SB. PMID: 3598605.
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    456. Immunoblotting of Creutzfeldt-Jakob disease prion proteins: host species-specific epitopes. Ann Neurol. 1987 Jun; 21(6):589-95. Bockman JM, Prusiner SB, Tateishi J, Kingsbury DT. PMID: 3300520.
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