Muscular dystrophies proteins evaluation by western blott and immunofluorescence

The muscular dystrophies are a diverse group of inherited muscle disorders characterized by progressive muscle weakness and wasting with characteristic histological abnormalities such as degeneration, necrosis, and regeneration of muscle fibers. Many muscle-wasting diseases are caused by defect in genes for muscle proteins. Most of these proteins appear to play a role in supporting the structure of muscle fibers, although some may play a role in the biochemical processes that go on in muscle fibers. DMD and DMB are X-linked allelic myopathies caused by dystrophin deficiency while LGMD 2A is caused by mutations in the calpain 3 gene. The aim of our studies is to evaluate the expression of dystrophin and calpain by Western blotting. The study of calpain 3 protein in muscle at this time can only be carried out by Western blotting since the antibodies that are available have no immunoreaction on the sections.