Intermediate filament-like protein syncoilin in normal and myopathic striated muscle.
The intermediate filament-like protein syncoilin is a member of the dystrophin protein complex, and links the complex to the cytoskeleton through binding alpha-dystrobrevin and desmin in muscle. Here, we identify further sites of syncoilin location in normal muscle: at the perinuclear space, myotendinous junction, and enrichment in the sarcolemma and sarcoplasm of oxidative muscle fibers in mice. To understand the importance of the dystrophin protein complex-syncoilin-cytoskeletal link and its implication to disease, we analyzed syncoilin in mice null for alpha-dystrobrevin (adbn-/-) and desmin (des-/-). Syncoilin was upregulated in dystrophic muscles of adbn-/- mice, without alteration in its subcellular location. In des-/- mice, syncoilin was severely reduced in skeletal muscle; lost from sarcomeric Z-lines and neuromuscular junctions, and redistributed from the sub-sarcolemmal cytoskeleton to the cytoplasm. The data show that absence of alpha-dystrobrevin or desmin leads to dynamic changes in syncoilin that may compensate for, or participate in, different muscle myopathies.
McCullagh
Karl J A
KJ
MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, South Parks Road, Oxford OX1 3QX, UK.
Edwards
Ben
B
Poon
Ellen
E
Lovering
Richard M
RM
Paulin
Denise
D
Davies
Kay E
KE
D000818:Animals; D003593:Cytoplasm / Q000378:metabolism / Q000648:ultrastructure; D003893:Desmin / Q000235:genetics; D049029:Dystrophin-Associated Proteins / Q000235:genetics; D007381:Intermediate Filament Proteins / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D046529:Microscopy, Electron, Transmission; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000648:ultrastructure; D009124:Muscle Proteins / Q000378:metabolism*; D054792:Muscle, Striated / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D009135:Muscular Diseases / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology; D009469:Neuromuscular Junction / Q000378:metabolism / Q000473:pathology; D012508:Sarcolemma / Q000378:metabolism / Q000648:ultrastructure; D012518:Sarcomeres / Q000378:metabolism / Q000473:pathology; D015854:Up-Regulation / Q000235:genetics
D003893:Desmin; D049029:Dystrophin-Associated Proteins; D007381:Intermediate Filament Proteins; D009124:Muscle Proteins; C503782:Sync protein, mouse; C098885:dystrobrevin