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MOLECULAR AND CLINICAL FEATURES OF KYPHOSCOLIOTIC EHLERS-DANLOS SYNDROME DUE TO LYSYL HYDROXYLASE 1 DEFICIENCY. Matrix Biol. 2026 Oct 06; 102055.
Syx D, Colman M, Malfait Z, De Smet G, de Hesselle HC, Panen E, Lachlan K, Ceylaner S, Björck E, Kindy AA, Elcioglu N, Kariminejad A, Demirdas S, Taga Y, Mizuno K, Naji S, Van Damme T, Symoens S, Ishikawa Y, Forneris F, Malfait F. PMID: 42838462.
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TANGO1L Is Required for Collagen Secretion in a Type-Dependent Manner and Shapes Collagen Proteostasis in the Endoplasmic Reticulum. Matrix Biol. 2026 Sep 16; 102052.
Ishikawa Y, Maeda M, Taga Y, Komatsu Y, Mizuno K, Saito K. PMID: 42749169.
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Fibulin-4 is required for the mechanical stability of tendons. Cell Tissue Res. 2025 Dec; 402(3):361-372.
Sasaki T, Jin K, Takimoto A, Taga Y, Ishikawa Y, Bächinger HP, Sakai T, Terabayashi T, Hanada K, Schlötzer-Schrehardt U, Shukunami C, Hiraki Y, Nakamura T, Yamamoto E. PMID: 41231259.
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AnimalsCells
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Evidence for the major role of PH4⍺EFB in the prolyl 4-hydroxylation of Drosophila collagen IV. Matrix Biol. 2025 11; 141:101-113.
Ishikawa Y, Toups MA, Elkrewi M, Zajac AL, Horne-Badovinac S, Matsubayashi Y. PMID: 40946811; PMCID: PMC12744885.
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HumansAnimalsCells
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Collagen Biosynthesis and Its Molecular Ensemble: What Remains Unexplored. Biochemistry. 2025 08 05; 64(15):3149-3155.
Ishikawa Y. PMID: 40637638; PMCID: PMC12329708.
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2 Fields:
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HumansAnimalsCells
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Collagen IV biosynthesis: Intracellular choreography of post-translational modifications. Matrix Biol. 2025 09; 140:59-77.
Ishikawa Y, Lennon R, Forneris F, Myllyharju J, Salo AM. PMID: 40618934.
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4 Fields:
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HumansAnimalsCells
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A multifunction murine Col4a1 allele reveals potential gene therapy parameters for Gould syndrome. J Cell Biol. 2025 06 02; 224(6).
Mao M, Ishikawa Y, Labelle-Dumais C, Wang X, Kuo YM, Gaffney UB, Smith ME, Abdala CN, Lebedev MD, Paradee WJ, Gould DB. PMID: 40279671; PMCID: PMC12029515.
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2 Fields:
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HumansAnimalsCells
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Biochemical characterization of collagen I in Warmblood Fragile Foal Syndrome horse lysyl hydroxylase 1 mutation. MicroPubl Biol. 2025; 2025.
Ishikawa Y, Tufa SF, Keene DR, Bächinger HP, Winand NJ. PMID: 39839713; PMCID: PMC11749069.
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Local Net Charge State of Collagen Triple Helix Is a Determinant of FKBP22 Binding to Collagen III. Int J Mol Sci. 2023 Oct 13; 24(20).
Ishikawa Y, Bonna A, Gould DB, Farndale RW. PMID: 37894834; PMCID: PMC10607241.
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1 Fields:
Translation:
Humans
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Lysyl hydroxylase 3-mediated post-translational modifications are required for proper biosynthesis of collagen α1α1α2(IV). J Biol Chem. 2022 12; 298(12):102713.
Ishikawa Y, Taga Y, Coste T, Tufa SF, Keene DR, Mizuno K, Tournier-Lasserve E, Gould DB. PMID: 36403858; PMCID: PMC9761383.
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9 Fields:
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HumansCells
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FK506-Binding Protein 11 Is a Novel Plasma Cell-Specific Antibody Folding Catalyst with Increased Expression in Idiopathic Pulmonary Fibrosis. Cells. 2022 04 14; 11(8).
Preisendörfer S, Ishikawa Y, Hennen E, Winklmeier S, Schupp JC, Knüppel L, Fernandez IE, Binzenhöfer L, Flatley A, Juan-Guardela BM, Ruppert C, Guenther A, Frankenberger M, Hatz RA, Kneidinger N, Behr J, Feederle R, Schepers A, Hilgendorff A, Kaminski N, Meinl E, Bächinger HP, Eickelberg O, Staab-Weijnitz CA. PMID: 35456020; PMCID: PMC9027113.
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10 Fields:
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HumansCells
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Sequence-dependent mechanics of collagen reflect its structural and functional organization. Biophys J. 2021 09 21; 120(18):4013-4028.
Al-Shaer A, Lyons A, Ishikawa Y, Hudson BG, Boudko SP, Forde NR. PMID: 34390685; PMCID: PMC8510973.
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15 Fields:
Translation:
Cells
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Type I and type V procollagen triple helix uses different subsets of the molecular ensemble for lysine posttranslational modifications in the rER. J Biol Chem. 2021 Jan-Jun; 296:100453.
Ishikawa Y, Taga Y, Zientek K, Mizuno N, Salo AM, Semenova O, Tufa SF, Keene DR, Holden P, Mizuno K, Gould DB, Myllyharju J, Bächinger HP. PMID: 33631195; PMCID: PMC7988497.
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15 Fields:
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AnimalsCells
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Aberrant binding of mutant HSP47 affects posttranslational modification of type I collagen and leads to osteogenesis imperfecta. PLoS Genet. 2021 02; 17(2):e1009339.
Syx D, Ishikawa Y, Gebauer J, Boudko SP, Guillemyn B, Van Damme T, D'hondt S, Symoens S, Nampoothiri S, Gould DB, Baumann U, Bächinger HP, Malfait F. PMID: 33524049; PMCID: PMC7877763.
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12 Fields:
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HumansCells
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The novel missense mutation Met48Lys in FKBP22 changes its structure and functions. Sci Rep. 2020 01 16; 10(1):497.
Ishikawa Y, Mizuno N, Holden P, Lim PJ, Gould DB, Rohrbach M, Giunta C, Bächinger HP. PMID: 31949249; PMCID: PMC6965642.
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4 Fields:
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HumansCells
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The HDAC3-SMARCA4-miR-27a axis promotes expression of the PAX3:FOXO1 fusion oncogene in rhabdomyosarcoma. Sci Signal. 2018 11 20; 11(557).
Bharathy N, Berlow NE, Wang E, Abraham J, Settelmeyer TP, Hooper JE, Svalina MN, Ishikawa Y, Zientek K, Bajwa Z, Goros MW, Hernandez BS, Wolff JE, Rudek MA, Xu L, Anders NM, Pal R, Harrold AP, Davies AM, Ashok A, Bushby D, Mancini M, Noakes C, Goodwin NC, Ordentlich P, Keck J, Hawkins DS, Rudzinski ER, Chatterjee B, Bächinger HP, Barr FG, Liddle J, Garcia BA, Mansoor A, Perkins TJ, Vakoc CR, Michalek JE, Keller C. PMID: 30459282; PMCID: PMC6432638.
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44 Fields:
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HumansAnimalsCells
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The endoplasmic reticulum-resident collagen chaperone Hsp47 interacts with and promotes the secretion of decorin, fibromodulin, and lumican. J Biol Chem. 2018 08 31; 293(35):13707-13716.
Ishikawa Y, Rubin K, Bächinger HP, Kalamajski S. PMID: 30002123; PMCID: PMC6120207.
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17 Fields:
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HumansAnimalsCells
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Type III collagen affects dermal and vascular collagen fibrillogenesis and tissue integrity in a mutant Col3a1 transgenic mouse model. Matrix Biol. 2018 09; 70:72-83.
D'hondt S, Guillemyn B, Syx D, Symoens S, De Rycke R, Vanhoutte L, Toussaint W, Lambrecht BN, De Paepe A, Keene DR, Ishikawa Y, Bächinger HP, Janssens S, Bertrand MJM, Malfait F. PMID: 29551664.
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34 Fields:
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HumansAnimalsCells
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Heat shock protein 47 and 65-kDa FK506-binding protein weakly but synergistically interact during collagen folding in the endoplasmic reticulum. J Biol Chem. 2017 10 20; 292(42):17216-17224.
Ishikawa Y, Holden P, Bächinger HP. PMID: 28860186; PMCID: PMC5655501.
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17 Fields:
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HumansAnimalsCells
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A Novel Antifibrotic Mechanism of Nintedanib and Pirfenidone. Inhibition of Collagen Fibril Assembly. Am J Respir Cell Mol Biol. 2017 07; 57(1):77-90.
Knüppel L, Ishikawa Y, Aichler M, Heinzelmann K, Hatz R, Behr J, Walch A, Bächinger HP, Eickelberg O, Staab-Weijnitz CA. PMID: 28257580.
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94 Fields:
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HumansCells
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Ziploc-ing the structure 2.0: Endoplasmic reticulum-resident peptidyl prolyl isomerases show different activities toward hydroxyproline. J Biol Chem. 2017 06 02; 292(22):9273-9282.
Ishikawa Y, Mizuno K, Bächinger HP. PMID: 28385890; PMCID: PMC5454108.
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PubMed Mentions:
15 Fields:
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HumansCells
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Intracellular mechanisms of molecular recognition and sorting for transport of large extracellular matrix molecules. Proc Natl Acad Sci U S A. 2016 10 11; 113(41):E6036-E6044.
Ishikawa Y, Ito S, Nagata K, Sakai LY, Bächinger HP. PMID: 27679847; PMCID: PMC5068301.
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66 Fields:
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HumansCells
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An essential receptor for adeno-associated virus infection. Nature. 2016 Feb 04; 530(7588):108-12.
Pillay S, Meyer NL, Puschnik AS, Davulcu O, Diep J, Ishikawa Y, Jae LT, Wosen JE, Nagamine CM, Chapman MS, Carette JE. PMID: 26814968; PMCID: PMC4962915.
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312 Fields:
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HumansAnimalsCells
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Ziploc-ing the structure: Triple helix formation is coordinated by rough endoplasmic reticulum resident PPIases. Biochim Biophys Acta. 2015 Oct; 1850(10):1983-93.
Ishikawa Y, Boudko S, Bächinger HP. PMID: 25583561.
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18 Fields:
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HumansAnimalsCells
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A substrate preference for the rough endoplasmic reticulum resident protein FKBP22 during collagen biosynthesis. J Biol Chem. 2014 Jun 27; 289(26):18189-201.
Ishikawa Y, Bächinger HP. PMID: 24821723; PMCID: PMC4140264.
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20 Fields:
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HumansCells
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Excessive transforming growth factor-β signaling is a common mechanism in osteogenesis imperfecta. Nat Med. 2014 Jun; 20(6):670-5.
Grafe I, Yang T, Alexander S, Homan EP, Lietman C, Jiang MM, Bertin T, Munivez E, Chen Y, Dawson B, Ishikawa Y, Weis MA, Sampath TK, Ambrose C, Eyre D, Bächinger HP, Lee B. PMID: 24793237; PMCID: PMC4048326.
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205 Fields:
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AnimalsCells
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Structure of human peptidyl-prolyl cis-trans isomerase FKBP22 containing two EF-hand motifs. Protein Sci. 2014 Jan; 23(1):67-75.
Boudko SP, Ishikawa Y, Nix J, Chapman MS, Bächinger HP. PMID: 24272907; PMCID: PMC3892300.
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13 Fields:
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HumansCells
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The concave face of decorin mediates reversible dimerization and collagen binding. J Biol Chem. 2013 Dec 06; 288(49):35526-33.
Islam M, Gor J, Perkins SJ, Ishikawa Y, Bächinger HP, Hohenester E. PMID: 24169694; PMCID: PMC3853298.
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22 Fields:
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HumansAnimalsCells
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An additional function of the rough endoplasmic reticulum protein complex prolyl 3-hydroxylase 1·cartilage-associated protein·cyclophilin B: the CXXXC motif reveals disulfide isomerase activity in vitro. J Biol Chem. 2013 Nov 01; 288(44):31437-46.
Ishikawa Y, Bächinger HP. PMID: 24043621; PMCID: PMC3814740.
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10 Fields:
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HumansAnimalsCells
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Posttranslational modifications in type I collagen from different tissues extracted from wild type and prolyl 3-hydroxylase 1 null mice. J Biol Chem. 2013 Aug 23; 288(34):24742-52.
Pokidysheva E, Zientek KD, Ishikawa Y, Mizuno K, Vranka JA, Montgomery NT, Keene DR, Kawaguchi T, Okuyama K, Bächinger HP. PMID: 23861401; PMCID: PMC3750170.
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28 Fields:
Translation:
AnimalsCells
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A molecular ensemble in the rER for procollagen maturation. Biochim Biophys Acta. 2013 Nov; 1833(11):2479-91.
Ishikawa Y, Bächinger HP. PMID: 23602968.
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97 Fields:
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HumansAnimalsCells
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Crystal structures of wild-type and mutated cyclophilin B that causes hyperelastosis cutis in the American quarter horse. BMC Res Notes. 2012 Nov 08; 5:626.
Boudko SP, Ishikawa Y, Lerch TF, Nix J, Chapman MS, Bächinger HP. PMID: 23137129; PMCID: PMC3522003.
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PubMed Mentions:
1 Fields:
Translation:
AnimalsCells
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Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding. J Biol Chem. 2012 Jun 22; 287(26):22253-65.
Ishikawa Y, Vranka JA, Boudko SP, Pokidysheva E, Mizuno K, Zientek K, Keene DR, Rashmir-Raven AM, Nagata K, Winand NJ, Bächinger HP. PMID: 22556420; PMCID: PMC3381186.
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29 Fields:
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AnimalsCells
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Prolyl 3-hydroxylase 1 null mice display abnormalities in fibrillar collagen-rich tissues such as tendons, skin, and bones. J Biol Chem. 2010 May 28; 285(22):17253-62.
Vranka JA, Pokidysheva E, Hayashi L, Zientek K, Mizuno K, Ishikawa Y, Maddox K, Tufa S, Keene DR, Klein R, Bächinger HP. PMID: 20363744; PMCID: PMC2878055.
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47 Fields:
Translation:
AnimalsCells
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Biochemical characterization of the prolyl 3-hydroxylase 1.cartilage-associated protein.cyclophilin B complex. J Biol Chem. 2009 Jun 26; 284(26):17641-7.
Ishikawa Y, Wirz J, Vranka JA, Nagata K, Bächinger HP. PMID: 19419969; PMCID: PMC2719403.
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PubMed Mentions:
66 Fields:
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AnimalsCells
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The rough endoplasmic reticulum-resident FK506-binding protein FKBP65 is a molecular chaperone that interacts with collagens. J Biol Chem. 2008 Nov 14; 283(46):31584-90.
Ishikawa Y, Vranka J, Wirz J, Nagata K, Bächinger HP. PMID: 18786928.
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PubMed Mentions:
74 Fields:
Translation:
AnimalsCells