منابع مشابه
Membrane 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 ...
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 ...
متن کاملThe Dystrophin-Glycoprotein Complex in the Prevention of Muscle Damage
Muscular dystrophies are genetically diverse but share common phenotypic features of muscle weakness, degeneration, and progressive decline in muscle function. Previous work has focused on understanding how disruptions in the dystrophin-glycoprotein complex result in muscular dystrophy, supporting a hypothesis that the muscle sarcolemma is fragile and susceptible to contraction-induced injury i...
متن کاملDystrophin-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...
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ژورنال
عنوان ژورنال: Current Opinion in Neurology
سال: 1995
ISSN: 1350-7540
DOI: 10.1097/00019052-199510000-00010