A component of the dystrophin-associated protein complex, dystrophin-associated glycoprotein, that is proteolytically processed to produce α-dystroglycan (624 aa) which is an extracellular laminin-binding glycoprotein that remains associated with transmembrane β-dystroglycan (242 aa) that associates with dystrophin in the cytoplasm. The actin cytoskeleton is linked, via dystrophin and dystroglycans, to the extracellular matrix. α-Dystroglycan also binds agrin and neurexin. Post-translational modification of α-dystroglycan by the LARGE acetylglucosaminyltransferase is essential for normal function and to prevent muscle degeneration. Hypoglycosylation of dystroglycan is a feature of Fukuyama muscular dystrophy and muscle–eye–brain disease.
Subjects: Medicine and Health.