Transcriptomics

Dataset Information

0

Gene Expression Profiling of PGC-1a KO mouse striata


ABSTRACT: Huntington’s Disease (HD) is an inherited neurodegenerative disease caused by a glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered energy metabolism have been implicated in HD pathogenesis. We report here that mutant huntingtin causes disruption of mitochondrial function by inhibiting expression of PGC-1a, a transcriptional coactivator that regulates several metabolic processes including mitochondrial biogenesis and respiration. Mutant huntingtin represses PGC-1a gene transcription by associating with the promoter and interfering with the CREB/TAF4-dependent transcriptional pathway critical for the regulation of PGC-1a gene expression. Crossbreeding of PGC-1a knockout mice with HD knock-in mice leads to increased neurodegeneration of striatal neurons and motor abnormalities in the HD mice. Importantly, expression of PGC-1a partially reverses the toxic effects of mutant huntingtin in cultured striatal neurons. Moreover, lentiviral-mediated delivery of PGC-1a in the striatum provides neuroprotection in the transgenic HD mice. These studies suggest a key role for PGC-1a in the control of energy metabolism in the early stages of HD pathogenesis. Keywords: PGC-1a, striatum

ORGANISM(S): Mus musculus

PROVIDER: GSE5786 | GEO | 2006/10/05

SECONDARY ACCESSION(S): PRJNA97141

REPOSITORIES: GEO

Dataset's files

Source:
Action DRS
Other
Items per page:
1 - 1 of 1

Similar Datasets

2007-11-13 | E-GEOD-5786 | biostudies-arrayexpress
2017-06-12 | GSE97101 | GEO
2016-02-16 | PXD003442 | Pride
2011-08-22 | E-GEOD-9038 | biostudies-arrayexpress
2011-08-23 | GSE9038 | GEO
2008-06-16 | E-GEOD-9804 | biostudies-arrayexpress
2008-06-16 | E-GEOD-9803 | biostudies-arrayexpress
2008-06-16 | E-GEOD-9375 | biostudies-arrayexpress
2008-04-10 | E-GEOD-10202 | biostudies-arrayexpress
2020-04-21 | GSE145879 | GEO