ANALYSIS AT THE MOLECULAR LEVEL OF THE INTERACTION BETWEEN ALPHA-DYSTROGLYCAN AND BETA-DYSTROGLYCAN AND ASSESSMENT OF ITS IMPLICATION FOR SKELETAL MUSCLE PHYSIOPATHOLOGY

All multicellular living organisms are surrounded by the extracellular matrix, a well-organized network formed by collagens, laminins and proteoglycans. Dystroglycan (DG) is localized at the cell surface and it is formed by two interacting subunits. DG represents one of the major adhesion complex that links the intracellular cytoskeleton to the extracellular matrix. In particular, in skeletal muscle DG interacts with a specialized form of extracellular matrix (basement membrane) ensuring stability to muscle fibers during the cycles of contraction and relaxation. Several muscular pathologies are characterized by an altered molecular structure of DG. Nonetheless, the details of DG involvement in neuromuscular diseases have yet to be fully clarified. The aim of our project is to shed new light on the molecular aspects of dystroglycan subunits crosstalk to better understand the role of DG in neuromuscular pathologies