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...

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Detalles Bibliográficos
Autores principales: Cabral, Wayne A., Perdivara, Irina, Weis, MaryAnn, Terajima, Masahiko, Blissett, Angela R., Chang, Weizhong, Perosky, Joseph E., Makareeva, Elena N., Mertz, Edward L., Leikin, Sergey, Tomer, Kenneth B., Kozloff, Kenneth M., Eyre, David R., Yamauchi, Mitsuo, Marini, Joan C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4072593/
https://www.ncbi.nlm.nih.gov/pubmed/24968150
http://dx.doi.org/10.1371/journal.pgen.1004465

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