Abnormal type I collagen post-translational modification and crosslinking in a cyclophilin B KO mouse model of recessive osteogenesis imperfecta.

Cyclophilin B (CyPB), encoded by PPIB, is an ER-resident peptidyl-prolyl cis-trans isomerase (PPIase) that functions independently and as a component of the collagen prolyl 3-hydroxylation complex. CyPB is proposed to be the major PPIase catalyzing the rate-limiting step in collagen folding. Mutatio...

Full description

Bibliographic Details
Main Authors: Wayne A Cabral, Irina Perdivara, MaryAnn Weis, Masahiko Terajima, Angela R Blissett, Weizhong Chang, Joseph E Perosky, Elena N Makareeva, Edward L Mertz, Sergey Leikin, Kenneth B Tomer, Kenneth M Kozloff, David R Eyre, Mitsuo Yamauchi, Joan C Marini
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-06-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC4072593?pdf=render