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.
MeSH terms
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Animals
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Down-Regulation
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Energy Metabolism
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Heat-Shock Proteins / genetics
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Heat-Shock Proteins / metabolism*
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Humans
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Huntingtin Protein
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Huntington Disease / etiology
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Huntington Disease / metabolism*
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Huntington Disease / pathology
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Mice
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Nerve Tissue Proteins / genetics
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Nerve Tissue Proteins / metabolism
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Neurodegenerative Diseases / metabolism*
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Neurodegenerative Diseases / pathology
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Nuclear Proteins / genetics
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Nuclear Proteins / metabolism
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Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha
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Trans-Activators / genetics
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Trans-Activators / metabolism*
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Transcription Factors / genetics
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Transcription Factors / metabolism*
Substances
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HTT protein, human
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Heat-Shock Proteins
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Htt protein, mouse
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Huntingtin Protein
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Nerve Tissue Proteins
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Nuclear Proteins
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PPARGC1A protein, human
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Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha
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Ppargc1a protein, mouse
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Trans-Activators
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Transcription Factors