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Article
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
This study finds a direct correlation between LARGE-glycan extension on dystroglycan and the protein’s capacity for extracellular matrix ligands; in regenerating mouse muscle, short LARGE-glycan polysaccharide...
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Article
An ancient retrotransposal insertion causes Fukuyama-type congenital muscular dystrophy
Fukuyama-type congenital muscular dystrophy (FCMD), one of the most common autosomal recessive disorders in Japan (incidence is 0.7–1.2 per 10,000 births), is characterized by congenital muscular dystrophy ass...
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Article
Overexpression of dystrophin in transgenic mdx mice eliminates dystrophic symptoms without toxicity
DUCHENNE and Becker muscular dystrophy (DMD and BMD) are X-linked recessive diseases caused by defective expression of dystrophin1,2. The mdx mouse, an animal model for DMD, has a mutation that eliminates express...
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Article
Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
DYSTROPHIN is associated with a complex of muscle membrane (sarcolemmal) glycoproteins that provide a linkage to the extracellular matrix protein, laminin1–8. The absence of dystrophin leads to a dramatic reducti...
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Article
Deficiency of the 50K dystrophin-associated glycoprotein in severe childhood autosomal recessive muscular dystrophy
X-LINKED recessive Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin, a membrane cytoskeletal protein1,2. Dystrophin is associated with a large oligomeric com-plex of sarcolemmal glycoprote...