Molecular analysis of the function of a muscle-specific caveolar protein

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Muscular dystrophy is one of the most common and most debilitating inherited diseases in humans. Muscle from patients with muscular dystrophy is highly susceptible to damage leading to muscle wasting. In order to understand muscular dystrophy and to design therapeutic treatments, it is essential that researchers gain a detailed understanding of the workings of the muscle cell surface membrane. Caveolae are small pits which cover the entire surface of the muscle fibre. The major protein of muscle caveolae is caveolin-3, and mutations in this protein cause some forms of muscular dystrophy. This proposal aims to examine the function of this protein using a number of strategies. The caveolin-3 gene will be disrupted in mice to produce mice which lack this protein. The muscle from these mice will then be examined to see what effect the lack of this protein has on muscle function and whether this muscle is similar to that in patients with muscular dystrophy. The muscle from these mice will then be used to design treatments for the disease. In addition, we will search for proteins which work together with caveolin-3 in order to understand how the protein works in healthy and diseased muscle.