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Cellular origins of auditory event-related potential deficits in Rett syndrome.


ABSTRACT: Dysfunction in sensory information processing is a hallmark of many neurological disorders, including autism spectrum disorders, schizophrenia and Rett syndrome (RTT). Using mouse models of RTT, a monogenic disorder caused by mutations in MECP2, we found that the large-scale loss of MeCP2 from forebrain GABAergic interneurons led to deficits in auditory event-related potentials and seizure manifestation, whereas the restoration of MeCP2 in specific classes of interneurons ameliorated these deficits.

SUBMITTER: Goffin D 

PROVIDER: S-EPMC4038660 | biostudies-literature | 2014 Jun

REPOSITORIES: biostudies-literature

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Cellular origins of auditory event-related potential deficits in Rett syndrome.

Goffin Darren D   Brodkin Edward S ES   Blendy Julie A JA   Siegel Steve J SJ   Zhou Zhaolan Z  

Nature neuroscience 20140428 6


Dysfunction in sensory information processing is a hallmark of many neurological disorders, including autism spectrum disorders, schizophrenia and Rett syndrome (RTT). Using mouse models of RTT, a monogenic disorder caused by mutations in MECP2, we found that the large-scale loss of MeCP2 from forebrain GABAergic interneurons led to deficits in auditory event-related potentials and seizure manifestation, whereas the restoration of MeCP2 in specific classes of interneurons ameliorated these defic  ...[more]

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