Dystroglycan in development and disease.
Our understanding of the structure and function of dystroglycan, a cell surface laminin/agrin receptor, has increased dramatically over the past two years. Structural studies, analysis of its binding partners, and targeted gene disruption have all contributed to the elucidation of the biological role of dystroglycan in development and disease. It is now apparent that dystroglycan plays a critical role in the pathogenesis of several muscular dystrophies and serves as a receptor for a human pathogen as well as being involved in early development, organ morphogenesis, and synaptogenesis.
Durbeej
M
M
Howard Hughes Medical Institute, Department of Physiology and Biophysics, Iowa City, IA 52242, USA. madeleine-durbeej@uiowa.edu
Henry
M D
MD
Campbell
K P
KP
D000818:Animals; D003598:Cytoskeletal Proteins; D049030:Dystroglycans; D006801:Humans; D007918:Leprosy / Q000209:etiology*; D008562:Membrane Glycoproteins; D051379:Mice; D009136:Muscular Dystrophies / Q000209:etiology*; D011956:Receptors, Cell Surface; D017978:Receptors, Growth Factor; D017474:Receptors, Laminin
D003598:Cytoskeletal Proteins; C483081:DAG1 protein, human; D008562:Membrane Glycoproteins; D011956:Receptors, Cell Surface; D017978:Receptors, Growth Factor; D017474:Receptors, Laminin; C080030:agrin receptor; D049030:Dystroglycans