Collagen VI Microfibril Formation Is Abolished by an α2(VI) von Willebrand Factor Type A Domain Mutation in a Patient with Ullrich Congenital Muscular Dystrophy

Collagen VI is an extracellular protein that most often contains the three genetically distinct polypeptide chains, α1(VI), α2(VI), and α3(VI), although three recently identified chains, α4(VI), α5(VI), and α6(VI), may replace α3(VI) in some situations. Each chain has a triple helix flanked by N- an...

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Detalhes bibliográficos
Main Authors: Tooley, Leona D., Zamurs, Laura K., Beecher, Nicola, Baker, Naomi L., Peat, Rachel A., Adams, Naomi E., Bateman, John F., North, Kathryn N., Baldock, Clair, Lamandé, Shireen R.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2010
Assuntos:
Glycobiology and Extracellular Matrices
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2963345/
https://ncbi.nlm.nih.gov/pubmed/20729548
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.152520
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