Dystrophin: Gene, Protein and Cell Biology

This book is concerned with advances in research on dystrophin, and how its absence gives rise to muscular dystrophy. It is the first book to address relationships between the molecular structure and function of dystrophin since the structure of the gene for this protein was elucidated in 1988. The volume covers recent advances in knowledge on the structure of both the dystrophin gene and its associated promoters, and on the protein itself. Functional interactions of dystrophin and its related proteins in the environment of the plasma membrane are a central feature of the book. Other aspects considered are the expression of the dystrophin complex in muscle, in the brain, and at the neuromuscular junction. The book concludes with discussions of muscle regeneration, gene therapy of Duchenne muscular dystrophy, and cellular approaches to the therapy of the disease.