Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy

Figure 1

Schematic representation of the dystrophin-glycoprotein complex (DGC) in skeletal muscle. This multiprotein complex anchors the extracellular matrix (ECM) to actin and other components of the cytoskeleton. O-Mannosylated -DG is a central component of this complex and serves as binding partner for a number of ECM proteins containing LG domains, such as laminin-211. -DG is a transmembrane protein and binds the actin cytoskeleton via the direct interaction with dystrophin. Other intracellular molecules being a part of, or associated with, DGC are dystrobrevin, syntrophins, and neural nitric oxide synthase (nNOS).