Altered synaptic clustering of GABAA receptors in mice lacking dystrophin (mdx mice)

摘要: Dystrophin is selectively localized in the postsynaptic density of neurons cerebral cortex, hippocampus and cerebellum. Here, we show by double-immunofluorescence staining that dystrophin extensively colocalized with GABAA receptor subunit clusters these brain regions. To determine relevance this observation, investigated mdx mice, which provide a model Duchenne muscular dystrophy, whether absence affects synaptic clustering receptors. A marked reduction number immunoreactive for alpha1 alpha2 subunits was observed in, respectively, cerebellum but not striatum, normally devoid dystrophin. Furthermore, alterations were accompanied change gephyrin staining, although majority These results indicate may play an important role or stabilization receptors subset central inhibitory synapses. deficits underlie cognitive impairment seen patients.