Article
Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy.
The Journal of clinical investigation - 1 Sept 2012
Beedle Aaron M, Turner Amy J, Saito Yoshiaki, Lueck John D, Foltz Steven J, Fortunato Marisa J, Nienaber Patricia M, Campbell Kevin P
Abstract excerpt
Dystroglycan is a transmembrane glycoprotein that links the extracellular basement membrane to cytoplasmic dystrophin. Disruption of the extensive carbohydrate structure normally present on α-dystroglycan causes an array of congenital and limb girdle muscular dystrophies known as dystroglycanopathies. The essential role of dystroglycan in development has hampered elucidation of the mechanisms underlying...
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