Article
Glycomic analyses of mouse models of congenital muscular dystrophy.
The Journal of biological chemistry - 17 Jun 2011
Stalnaker Stephanie H, Aoki Kazuhiro, Lim Jae-Min, Porterfield Mindy, Liu Mian, Satz Jakob S, Buskirk Sean, Xiong Yufang, Zhang Peng, Campbell Kevin P, Hu Huaiyu, Live David, Tiemeyer Michael, Wells Lance
Abstract excerpt
Dystroglycanopathies are a subset of congenital muscular dystrophies wherein α-dystroglycan (α-DG) is hypoglycosylated. α-DG is an extensively O-glycosylated extracellular matrix-binding protein and a key component of the dystrophin-glycoprotein complex. Previous studies have shown α-DG to be post-translationally modified by both O-GalNAc- and O-mannose-initiated glycan structures. Mutations in defined or...
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