Abnormal membranerepair as a new concept for muscular dystrophy and search for new disease genes

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Anti-dysferlin antibodies and a method to isolate dysferlin-containing vesicle were established, which enables us to analyze components of the dysferlin-containing membranes. Proteins up-regulated correlatively to disease severity of muscular dystrophy were identified. Analyses of double mutant mice of dysferlin and other muscular dystrophy mice showed that dysferlin-deficiency led more severe muscular dystrophic phenotype. These results will contribute to deeper understanding of muscular dystrophy pathogenesis.