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Structure of a collagen VI α3 chain VWA domain array: adaptability and functional implications of myopathy causing mutations

Collagen VI is a ubiquitous heterotrimeric protein of the extracellular matrix (ECM) that plays an essential role in the proper maintenance of skeletal muscle. Mutations in collagen VI lead to a spectrum of congenital myopathies, from the mild Bethlem myopathy to the severe Ullrich congenital muscul...

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Detaylı Bibliyografya
Yayımlandı:J Biol Chem
Asıl Yazarlar: Solomon-Degefa, Herimela, Gebauer, Jan M., Jeffries, Cy M., Freiburg, Carolin D., Meckelburg, Patrick, Bird, Louise E., Baumann, Ulrich, Svergun, Dmitri I., Owens, Raymond J., Werner, Jörn M., Behrmann, Elmar, Paulsson, Mats, Wagener, Raimund
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: American Society for Biochemistry and Molecular Biology 2020
Konular:
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC7476709/
https://ncbi.nlm.nih.gov/pubmed/32719005
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.RA120.014865
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