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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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: American Society for Biochemistry and Molecular Biology 2008
Assuntos:
Protein Structure and Folding
Acceso en liña: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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