PGC-1alpha, a new therapeutic target in Huntington's disease?
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.
McGill
Jetta K
JK
Department of Neurology, Cornell Medical Center, New York, NY 10021, USA.
Beal
M Flint
MF
D000818:Animals; D015536:Down-Regulation; D004734:Energy Metabolism; D006360:Heat-Shock Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D051379:Mice; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism; D019636:Neurodegenerative Diseases / Q000378:metabolism* / Q000473:pathology; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism; D000071248:Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; D015534:Trans-Activators / Q000235:genetics / Q000378:metabolism*; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism*
C086055:HTT protein, human; D006360:Heat-Shock Proteins; C506812:Htt protein, mouse; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; C486497:PPARGC1A protein, human; D000071248:Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; C485290:Ppargc1a protein, mouse; D015534:Trans-Activators; D014157:Transcription Factors