Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome

Dysfunction in sensory information processing is a hallmark of many neurological disorders including autism spectrum disorders (ASDs), schizophrenia and Rett syndrome (RTT)(1). Using mouse models of RTT, a monogenic disorder caused by mutations in MECP2(2), we demonstrate that the large scale loss o...

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Detalles Bibliográficos
Autores principales: Goffin, Darren, Brodkin, Edward S., Blendy, Julie A., Siegel, Steve J., Zhou, Zhaolan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2014
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4038660/
https://www.ncbi.nlm.nih.gov/pubmed/24777420
http://dx.doi.org/10.1038/nn.3710
Descripción
Sumario:Dysfunction in sensory information processing is a hallmark of many neurological disorders including autism spectrum disorders (ASDs), schizophrenia and Rett syndrome (RTT)(1). Using mouse models of RTT, a monogenic disorder caused by mutations in MECP2(2), we demonstrate that the large scale loss of MeCP2 from forebrain GABAergic interneurons leads to deficits in auditory event-related potentials (ERPs) and seizure manifestation; but the restoration of MeCP2 in specific classes of interneurons ameliorates these deficits.

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