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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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
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author Goffin, Darren
Brodkin, Edward S.
Blendy, Julie A.
Siegel, Steve J.
Zhou, Zhaolan
author_facet Goffin, Darren
Brodkin, Edward S.
Blendy, Julie A.
Siegel, Steve J.
Zhou, Zhaolan
author_sort Goffin, Darren
collection PubMed
description 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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spelling pubmed-40386602014-12-01 Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome Goffin, Darren Brodkin, Edward S. Blendy, Julie A. Siegel, Steve J. Zhou, Zhaolan Nat Neurosci Article 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. 2014-04-28 2014-06 /pmc/articles/PMC4038660/ /pubmed/24777420 http://dx.doi.org/10.1038/nn.3710 Text en http://www.nature.com/authors/editorial_policies/license.html#terms Users may view, print, copy, and download text and data-mine the content in such documents, for the purposes of academic research, subject always to the full Conditions of use:http://www.nature.com/authors/editorial_policies/license.html#terms
spellingShingle Article
Goffin, Darren
Brodkin, Edward S.
Blendy, Julie A.
Siegel, Steve J.
Zhou, Zhaolan
Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome
title Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome
title_full Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome
title_fullStr Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome
title_full_unstemmed Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome
title_short Cellular Origins of Auditory Event-Related Potential Deficits in Rett Syndrome
title_sort cellular origins of auditory event-related potential deficits in rett syndrome
topic Article
url 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
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