Structural Basis of Dystroglycan Function and the Pathogenesis of Muscular Dystrophy
نویسندگان
چکیده
منابع مشابه
Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy
In skeletal muscle, dystroglycan (DG) is the central component of the dystrophin-glycoprotein complex (DGC), a multimeric protein complex that ensures a strong mechanical link between the extracellular matrix and the cytoskeleton. Several muscular dystrophies arise from mutations hitting most of the components of the DGC. Mutations within the DG gene (DAG1) have been recently associated with tw...
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Muscular dystrophy is frequently caused by disruption of the dystrophin-glycoprotein complex (DGC), which links muscle cells to the extracellular matrix. Dystroglycan, a central component of the DGC, serves as a laminin receptor via its extracellular alpha subunit, and interacts with dystrophin (and thus the actin cytoskeleton) through its integral membrane beta subunit. We have removed the fun...
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ژورنال
عنوان ژورنال: The FASEB Journal
سال: 2021
ISSN: 0892-6638,1530-6860
DOI: 10.1096/fasebj.2021.35.s1.00008