X-linked vacuolated myopathy: membrane attack complex deposition on muscle fiber membranes with calcium accumulation on sarcolemma.
Cytochemical localization of calcium on muscle fibers from patients with X-linked vacuolated myopathy demonstrated strong sarcolemmal or vacuolar calcium deposits in histologically abnormal muscle fibers. All membrane attack complex-positive fibers demonstrated such calcium accumulation. Total content of calcium was significantly elevated in muscles from patients with X-linked vacuolated myopathy compared to control muscles. Taken together, these results suggest that calcium accumulation on sarcolemma could be probably secondary to membrane attack complex deposition on the cell surface.
Louboutin
J P
JP
Laboratory of Cellular and Molecular Physiopathology and Pharmacology, CJF INSERM, Hopital Guillaume and René Laënnec, Nantes, France.
Villanova
M
M
Lucas-Héron
B
B
Fardeau
M
M
D002118:Calcium / Q000378:metabolism*; D008040:Genetic Linkage; D008854:Microscopy, Electron; D018485:Muscle Fibers, Skeletal / Q000648:ultrastructure*; D009135:Muscular Diseases / Q000235:genetics* / Q000473:pathology; D012508:Sarcolemma / Q000648:ultrastructure*; D014463:Ultrasonography; D014617:Vacuoles / Q000000981:diagnostic imaging; D014960:X Chromosome
D002118:Calcium