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Duchenne muscular dystrophy is a lethal genetic disease of childhood causedby primary abnormalities in the gene coding for the membrane cytoskeletal proteindystrophin. The mdx mouse is an established animal model of various aspects ofX-linked muscular dystrophy and is widely used for studying fundamental mechanismsof dystrophinopathy and testing novel therapeutic approaches to treat one of themost frequent gender-specific diseases in humans. In order to determine globalchanges in the muscle proteome with the progressive deterioration of mdx tissuewith age, we have characterized diaphragm muscle from mdx mice at three ages (8-weeks,12-months and 22-months) using mass spectrometry-based proteomics. Altered expressionlevels in diaphragm of 8-week vs. 22-month mice were shown to occur in 11 muscle-associatedproteins. Aging in the mdx diaphragm seems to be associated with a drastic increasein the extracellular matrix proteins, collagen and dermatopontin, the molecularchaperone αB-crystallin, and the intermediate filament protein vimentin, suggestingincreased accumulation of connective tissue, an enhanced cellular stress responseand compensatory stabilization of the weakened membrane cytoskeleton. These proteomicfindings establish the aged mdx diaphragm as an excellent model system for studyingsecondary effects of dystrophin deficiency in skeletal muscle tissue.

international journal of molecular medicine

Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy