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

Autor: Mats Paulsson, Jörn M. Werner, Raimund Wagener, Dmitri I. Svergun, Carolin D. Freiburg, Herimela Solomon-Degefa, Ulrich Baumann, Cy M. Jeffries, Louise E. Bird, Jan M. Gebauer, Elmar Behrmann, Patrick Meckelburg, Raymond J. Owens

Publikováno v: 'Journal of Biological Chemistry ', vol: 295, pages: 12755-12771 (2020)
The Journal of Biological Chemistry
JBC papers in press 295(36), 12755-12771 (2020). doi:10.1074/jbc.RA120.014865
J Biol Chem

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 Bethle

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https://epn-library.esrf.fr/flora/jsp/index_view_direct_anonymous.jsp?record=doc:PUB_ESRF:55852