Defective C-propeptides of the Proα2(I) Chain of Type I Procollagen Impede Molecular Assembly and Result in Osteogenesis Imperfecta

Type I procollagen is a heterotrimer composed of two proα1(I) chains and one proα2(I) chain, encoded by the COL1A1 and COL1A2 genes, respectively. Mutations in these genes usually lead to dominantly inherited forms of osteogenesis imperfecta (OI) by altering the triple helical domains, but a few aff...

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Detalhes bibliográficos
Main Authors: Pace, James M., Wiese, Mary, Drenguis, Andrea S., Kuznetsova, Natalia, Leikin, Sergey, Schwarze, Ulrike, Chen, Diana, Mooney, Suzanne H., Unger, Sheila, Byers, Peter H.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2008
Assuntos:
Protein Structure and Folding
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2414283/
https://ncbi.nlm.nih.gov/pubmed/18375391
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M801982200
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