Targeting dystroglycan in the brain.
The dystrophin-glycoprotein complex is a multisubunit complex that connects the extracellular matrix components to the cytoskeletal matrix of muscle fiber cells and is required for muscle integrity. Mutations in this complex are associated with muscular dystrophy. Although the role of dystroglycan has been explored mainly in the context of muscle, recent work has also demonstrated a novel role for dystroglycan in the CNS and thus provides potential insights into the brain abnormalities associated with some forms of muscular dystrophy.
Montanaro
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F
Children's Hospital, Harvard University and Howard Hughes Medical Institute, Boston, MA 02446, USA.
Carbonetto
S
S
D000818:Animals; D001485:Basement Membrane / Q000378:metabolism; D001923:Brain Chemistry / Q000502:physiology*; D002465:Cell Movement / Q000502:physiology; D003598:Cytoskeletal Proteins / Q000378:metabolism / Q000502:physiology*; D049030:Dystroglycans; D006801:Humans; D008562:Membrane Glycoproteins / Q000378:metabolism / Q000502:physiology*; D018482:Muscle, Skeletal / Q000502:physiology; D009474:Neurons / Q000502:physiology; D009435:Synaptic Transmission / Q000502:physiology
D003598:Cytoskeletal Proteins; C483081:DAG1 protein, human; D008562:Membrane Glycoproteins; D049030:Dystroglycans