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Title: Control of Huntington's Disease-Associated Phenotypes by the Striatum-Enriched Transcription Factor Foxp2
Authors: Hachigian, Lea J.
Carmona, Vitor 
Fenster, Robert J.
Kulicke, Ruth
Heilbut, Adrian
Sittler, Annie
Almeida, Luís Pereira de 
Mesirov, Jill P.
Gao, Fan
Kolaczyk, Eric D.
Heiman, Myriam
Keywords: Foxp2; Huntington’s disease; corticostriatal synapse; striatum
Issue Date: 5-Dec-2017
Publisher: Elsevier
Project: Lock/Chin family 
Latham family 
JPB Foundation 
NSF predoctoral fellowship 
Serial title, monograph or event: Cell Reports
Volume: 21
Issue: 10
Abstract: Alteration of corticostriatal glutamatergic function is an early pathophysiological change associated with Huntington's disease (HD). The factors that regulate the maintenance of corticostriatal glutamatergic synapses post-developmentally are not well understood. Recently, the striatum-enriched transcription factor Foxp2 was implicated in the development of these synapses. Here, we show that, in mice, overexpression of Foxp2 in the adult striatum of two models of HD leads to rescue of HD-associated behaviors, while knockdown of Foxp2 in wild-type mice leads to development of HD-associated behaviors. We note that Foxp2 encodes the longest polyglutamine repeat protein in the human reference genome, and we show that it can be sequestered into aggregates with polyglutamine-expanded mutant Huntingtin protein (mHTT). Foxp2 overexpression in HD model mice leads to altered expression of several genes associated with synaptic function, genes that present additional targets for normalization of corticostriatal dysfunction in HD.
ISSN: 22111247
DOI: 10.1016/j.celrep.2017.11.018
Rights: openAccess
Appears in Collections:I&D CNC - Artigos em Revistas Internacionais

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