Essential roles of the dystrophin-glycoprotein complex in different cardiac pathologies
نویسندگان
چکیده
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...
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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 کاملMaturation and Maintenance of the Neuromuscular Synapse Genetic Evidence for Roles of the Dystrophin–Glycoprotein Complex
The dystrophin-glycoprotein complex (DGC) links the cytoskeleton of muscle fibers to their extracellular matrix. Using knockout mice, we show that a cytoplasmic DGC component, alpha-dystrobrevin (alpha-DB), is dispensable for formation of the neuromuscular junction (NMJ) but required for maturation of its postsynaptic apparatus. We also analyzed double and triple mutants lacking other cytoskele...
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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: molecular organization and critical roles in skeletal muscle.
Recent molecular and biochemical studies have disclosed the detailed molecular organization of the dystrophin-glycoprotein complex, which links the cytoskeleton to the extracellular matrix. Defects in several components of this complex cause different types of muscular dystrophy. This glycoprotein complex is also involved in clustering and anchoring acetylcholine receptors at the postsynaptic m...
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ژورنال
عنوان ژورنال: Advances in Medical Sciences
سال: 2021
ISSN: 1896-1126
DOI: 10.1016/j.advms.2020.12.004