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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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Bibliografiske detaljer
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.
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Biochemistry and Molecular Biology 2008
Fag:
Online adgang: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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