Dystrophin-Glycoprotein Complex: Post-translational Processing and Dystroglycan Function
نویسندگان
چکیده
منابع مشابه
Dystrophin-glycoprotein complex: post-translational processing and dystroglycan function.
The dystrophin-glycoprotein complex (DGC) is a multimeric transmembrane protein complex first isolated from skeletal muscle membranes (1). The central protein of the DGC is dystroglycan (Fig. 1). In addition to skeletal muscle, dystroglycan is strongly expressed in heart and smooth muscle, as well as many non-muscle tissues including brain and peripheral nerve. In vertebrates, dystroglycan is g...
متن کاملDystroglycan and muscular dystrophies related to the dystrophin-glycoprotein complex.
Dystroglycan (DG) is an adhesion molecule composed of two subunits, alpha and beta, that are produced by the post-translational cleavage of a single precursor molecule. DG is a pivotal component of the dystrophin-glycoprotein complex (DGC), which connects the extracellular matrix to the cytoskeleton in skeletal muscle and many other tissues. Some muscular dystrophies are caused by mutations of ...
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Dystroglycan is a cell-surface matrix receptor that requires LARGE-dependent glycosylation for laminin binding. Although the interaction of dystroglycan with laminin has been well characterized, less is known about the role of dystroglycan glycosylation in the binding and assembly of perlecan. We report reduced perlecan-binding activity and mislocalization of perlecan in the LARGE-deficient Lar...
متن کاملMembrane organization of the dystrophin-glycoprotein complex.
The stoichiometry, cellular location, glycosylation, and hydrophobic properties of the components in the dystrophin-glycoprotein complex were examined. The 156, 59, 50, 43, and 35 kd dystrophin-associated proteins each possess unique antigenic determinants, enrich quantitatively with dystrophin, and were localized to the skeletal muscle sarcolemma. The 156, 50, 43, and 35 kd dystrophin-associat...
متن کاملDiversity of the Brain Dystrophin-Glycoprotein Complex
Duchenne muscular dystrophy (DMD), the most common inherited neuromuscular disorder, is characterized by progressive muscle wasting and weakness. One third of Duchenne patients suffer a moderate to severe, nonprogressive form of mental retardation. Mutations in the DMD gene are thought to be responsible, with the shorter isoforms of dystrophin implicated in its molecular brain pathogenesis. It ...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2003
ISSN: 0021-9258
DOI: 10.1074/jbc.r200031200