Article
[Fukuyama congenital muscular dystrophy and related alpha-dystroglycanopathies].
Brain and nerve = Shinkei kenkyu no shinpo - 1 Oct 2008
Murakami Terumi, Nishino Ichizo
Abstract excerpt
Alpha-dystroglycan (alpha-DG) is a glycoprotein that binds to laminin in the basal lamina and helps provide mechanical support. A group of muscular dystrophies are caused by glycosylation defects of alpha-DG and are hence collectively called alpha-dystroglycanopathy (alpha-DGP). Alpha-DGP is clinically characterized by a combination of muscular dystrophies, structural brain anomalies, and ocular involvement. So...
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