Please join us for our next Pharmacology Seminar Series: “Synaptic defects in mouse models of dystroglycanopathy”

Abstract: Dystroglycan is a heavily glycosylated transmembrane protein that links the extracellular environment to the actin cytoskeleton. Defects in dystroglycan function cause a form of congenital muscular dystrophy (dystroglycanopathy) that is often accompanied by neurological dysfunction. Using multiple mouse models of dystroglycanopathy, we have identified a critical role for dystroglycan in regulating the formation and maintenance of subsets of inhibitory synapses in the brain. Our results show that dystroglycan's synaptic function requires both proper glycosylation and its intracellular domain, and that the degree of dysfunctional glycosylation correlates with synaptic defects and seizure susceptibility