Review
doi: 10.1016/j.cell.2006.10.023.
PGC-1alpha, a new therapeutic target in Huntington's disease?
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- PMID: 17081970
- DOI: 10.1016/j.cell.2006.10.023
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Review
PGC-1alpha, a new therapeutic target in Huntington's disease?
Cell.
.
Free article
Abstract
The coactivator PGC-1alpha is a key regulator of mitochondrial biogenesis and respiration, mediating expression of several transcription factors required for these programs. Three new studies reveal that PGC-1alpha expression is downregulated in patients with Huntington's disease (HD) and in several animal models of this neurodegenerative disorder, implicating PGC-1alpha in HD pathogenesis and providing a connection between impaired energy metabolism and neurodegeneration.
Comment on
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Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.Cell. 2006 Oct 6;127(1):59-69. doi: 10.1016/j.cell.2006.09.015. Cell. 2006. PMID: 17018277
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Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators.Cell. 2006 Oct 20;127(2):397-408. doi: 10.1016/j.cell.2006.09.024. Cell. 2006. PMID: 17055439
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