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  1. No Access

    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...

    Matthew M. Goddeeris, Biming Wu, David Venzke, Takako Yoshida-Moriguchi… in Nature (2013)

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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...

    Kazuhiro Kobayashi, Yutaka Nakahori, Masashi Miyake, Kiichiro Matsumura… in Nature (1998)

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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...

    Gregory A. Cox, Neil M. Cole, Kiichiro Matsumura, Stephanie F. Phelps… in Nature (1993)

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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...

    Kiichiro Matsumura, James M. Ervasti, Kay Ohlendieck, Steven D. Kahl… in Nature (1992)

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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...

    Kiichiro Matsumura, Fernando M. S. Tomé, Huguette Collin, Kemal Azibi… in Nature (1992)