A Chaperone Complex Formed by HSP47, FKBP65, and BiP Modulates Telopeptide Lysyl Hydroxylation of Type I Procollagen

被引：57

作者：

Duran, Ivan ^{[1
,2
]}

Martin, Jorge H. ^{[1
]}

Weis, Mary Ann ^{[3
]}

Krejci, Pavel ^{[4
]}

Konik, Peter ^{[5
]}

Li, Bing ^{[6
]}

Alanay, Yasemin ^{[7
]}

Lietman, Caressa ^{[8
]}

Lee, Brendan ^{[8
,9
]}

Eyre, David ^{[3
]}

Cohn, Daniel H. ^{[1
,6
]}

Krakow, Deborah ^{[10
,11
]}

机构：

[1] Univ Calif Los Angeles, David Geffen Sch Med, Dept Orthopaed Surg, Los Angeles, CA 90095 USA

[2] Univ Malaga, Networking Res Ctr Bioengn Biomat & Nanomed CIBER, Malaga, Spain

[3] Univ Washington, Dept Orthopaed & Sports Med, Seattle, WA 98195 USA

[4] Masaryk Univ, St Annes Univ Hosp, Fac Med, Int Clin Res Ctr,Dept Biol, Brno, Czech Republic

[5] Univ South Bohemia, Fac Sci, Ceske Budejovice, Czech Republic

[6] Univ Calif Los Angeles, Dept Mol Cell & Dev Biol, Los Angeles, CA USA

[7] Acibadem Univ, Sch Med, Dept Pediat, Pediat Genet Unit, Istanbul, Turkey

[8] Baylor Coll Med, Dept Mol & Human Genet, Houston, TX 77030 USA

[9] Howard Hughes Med Inst, Chevy Chase, MD USA

[10] Univ Calif Los Angeles, David Geffen Sch Med, Dept Human Genet, Los Angeles, CA 90095 USA

[11] Univ Calif Los Angeles, David Geffen Sch Med, Dept Obstet & Gynecol, Los Angeles, CA 90095 USA

来源：

JOURNAL OF BONE AND MINERAL RESEARCH | 2017年 / 32卷 / 06期

基金：

美国国家卫生研究院;

关键词：

RECESSIVE OSTEOGENESIS IMPERFECTA; CARBOXYL-TERMINAL PROPEPTIDE; PYRIDINOLINE CROSS-LINKS; BRUCK-SYNDROME; ENDOPLASMIC-RETICULUM; MOLECULAR CHAPERONE; PROXIMITY LIGATION; COLLAGEN; MUTATIONS; FIBROSIS;

D O I：

10.1002/jbmr.3095

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Lysine hydroxylation of type I collagen telopeptides varies from tissue to tissue, and these distinct hydroxylation patterns modulate collagen cross-linking to generate a unique extracellular matrix. Abnormalities in these patterns contribute to pathologies that include osteogenesis imperfecta (OI), fibrosis, and cancer. Telopeptide procollagen modifications are carried out by lysyl hydroxylase 2 (LH2); however, little is known regarding how this enzyme regulates hydroxylation patterns. We identified an ER complex of resident chaperones that includes HSP47, FKBP65, and BiP regulating the activity of LH2. Our findings show that FKBP65 and HSP47 modulate the activity of LH2 to either favor or repress its activity. BiP was also identified as a member of the complex, playing a role in enhancing the formation of the complex. This newly identified ER chaperone complex contributes to our understanding of how LH2 regulates lysyl hydroxylation of type I collagen C-telopeptides to affect the quality of connective tissues. (C) 2017 American Society for Bone and Mineral Research.

引用

页码：1309 / 1319

页数：11

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