Intermediate filament-like protein syncoilin in normal and myopathic striated muscle

摘要: Abstract The intermediate filament-like protein syncoilin is a member of the dystrophin complex, and links complex to cytoskeleton through binding α-dystrobrevin desmin in muscle. Here, we identify further sites location normal muscle: at perinuclear space, myotendinous junction, enrichment sarcolemma sarcoplasm oxidative muscle fibers mice. To understand importance complex-syncoilin-cytoskeletal link its implication disease, analyzed mice null for ( adbn −/−) des −/−). Syncoilin was upregulated dystrophic muscles −/− mice, without alteration subcellular location. In severely reduced skeletal muscle; lost from sarcomeric Z-lines neuromuscular junctions, redistributed sub-sarcolemmal cytoplasm. data show that absence or leads dynamic changes may compensate for, participate in, different myopathies.