Dystroglycan (DG) is a cell surface receptor for several extracellular matrix molecules including laminins, Agrin and Perlecan. Dystroglycan function is required for the formation of basement membranes in early development and the organization of Laminin on the cell surface. α-dystroglycan is a membrane- associated, extracellular glycoprotein that is anchored to the cell-membrane by binding to the transmembrane glycoprotein α-dystroglycan to form an α/β-dystroglycan-complex. Additionally, dystroglycan is part of a multimolecular complex, where it associates with dystrophin, at the sarcolemma, to form the dystrophin-associated protein complex, or with utrophin, at the neuromuscular junction, to form the utrophin-associated protein complex. Dystroglycan is also thought to participate in the clustering of nicotinic acetylcholine receptors at the neuromuscular junction.