research data . Dataset . 2016

Abnormal Type I Collagen Post-translational Modification and Crosslinking in a Cyclophilin B KO Mouse Model of Recessive Osteogenesis Imperfecta

A. Cabral, Wayne; Perdivara, Irina; Weis, MaryAnn; Terajima, Masahiko; R. Blissett, Angela; Chang, Weizhong; E. Perosky, Joseph; N. Makareeva, Elena; Mertz, Edward L.; Leikin, Sergey; ...
  • Published: 01 Jan 2016
  • Publisher: Figshare
Abstract
<div><p>Cyclophilin B (CyPB), encoded by <i>PPIB</i>, is an ER-resident peptidyl-prolyl <i>cis-trans</i> 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. Mutations in <i>PPIB</i> cause recessively inherited osteogenesis imperfecta type IX, a moderately severe to lethal bone dysplasia. To investigate the role of CyPB in collagen folding and post-translational modifications, we generated <i>Ppib<sup>−/−</sup></i> mice that recapitulate the OI phenotype. Knock-out (KO) mice are small, with reduced femoral a...
Subjects
free text keywords: Biological Sciences, Biochemistry, genetics, Clinical genetics, Model organisms, collagen, post-translational, modification, crosslinking, cyclophilin, ko, recessive, osteogenesis
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Dataset . 2016
Provider: figshare
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