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, 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.
Department of Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Darren
D
Goffin
Department of Psychiatry, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Edward S
ES
Brodkin
Department of Pharmacology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Julie A
JA
Blendy
Department of Psychiatry, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Steve J
SJ
Siegel
Department of Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Zhaolan
Z
Zhou
D000818:Animals; D005072:Evoked Potentials, Auditory / Q000502:physiology*; D007395:Interneurons / Q000473:pathology* / Q000502:physiology; D008297:Male; D051783:Methyl-CpG-Binding Protein 2 / Q000172:deficiency / Q000235:genetics*; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D016548:Prosencephalon / Q000473:pathology* / Q000502:physiology; D015518:Rett Syndrome / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology*
C497393:Mecp2 protein, mouse; D051783:Methyl-CpG-Binding Protein 2