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X-linked vacuolated myopathy: membrane attack complex deposition on muscle fiber membranes with calcium accumulation
J P Louboutin1, M Villanova, B Lucas-Héron
1Laboratory of Cellular and Molecular Physiopathology and Pharmacology, CJF INSERM, Hopital Guillaume and René Laënnec, Nantes, France.
Abstract:
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.