PGC-1α at the intersection of bioenergetics regulation and neuron function: From Huntington’s disease to Parkinson’s disease and beyond
Identifieur interne : 000200 ( Main/Exploration ); précédent : 000199; suivant : 000201PGC-1α at the intersection of bioenergetics regulation and neuron function: From Huntington’s disease to Parkinson’s disease and beyond
Auteurs : Taiji Tsunemi [États-Unis] ; Albert R. La Spada [États-Unis]Source :
- Progress in neurobiology [ 0301-0082 ] ; 2011.
Abstract
Neurons are specialized cells with unique features, including a constant high demand for energy. Mitochondria satisfy this constant demand, and are emerging as a central target for dysfunction in neurodegenerative disorders, such as Huntington’s disease (HD) and Parkinson’s disease. PPARγ co-activator-1α (PGC-1α) is a transcription co-activator for nuclear receptors such as the PPARs, and thereby coordinates a number of gene expression programs to promote mitochondrial biogenesis and oxidative phosphorylation. Studies of PGC-1α knock-out mice have yielded important insights into the role of PGC-1α in normal nervous system function and potentially neurological disease. HD is caused by a polyglutamine repeat expansion in the huntingtin protein, and decades of work have established mitochondrial dysfunction as a key feature of HD pathogenesis. However, after the discovery of the HD gene, numerous reports produced strong evidence for altered transcription in HD. In 2006, a series of studies revealed that PGC-1α transcription interference contributes to HD neurodegeneration, linking the nuclear transcriptionopathy with the mitochondrial dysfunction. Subsequent work has strengthened this view, and further extended the role of PGC-1α within the CNS. Within the last year, studies of Parkinson’s disease, another involuntary movement disorder long associated with mitochondrial dysfunction, have shown that PGC-1α dysregulation is contributing to its pathogenesis. As PGC-1α is likely also important for aging, a process with considerable relevance to neuron function, translational studies aimed at developing therapies based upon the PGC-1α pathway as a high priority target are underway.
Url:
DOI: 10.1016/j.pneurobio.2011.10.004
PubMed: 22100502
PubMed Central: 3506171
Affiliations:
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Le document en format XML
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<front><div type="abstract" xml:lang="en"><p id="P1">Neurons are specialized cells with unique features, including a constant high demand for energy. Mitochondria satisfy this constant demand, and are emerging as a central target for dysfunction in neurodegenerative disorders, such as Huntington’s disease (HD) and Parkinson’s disease. PPARγ co-activator-1α (PGC-1α) is a transcription co-activator for nuclear receptors such as the PPARs, and thereby coordinates a number of gene expression programs to promote mitochondrial biogenesis and oxidative phosphorylation. Studies of PGC-1α knock-out mice have yielded important insights into the role of PGC-1α in normal nervous system function and potentially neurological disease. HD is caused by a polyglutamine repeat expansion in the huntingtin protein, and decades of work have established mitochondrial dysfunction as a key feature of HD pathogenesis. However, after the discovery of the HD gene, numerous reports produced strong evidence for altered transcription in HD. In 2006, a series of studies revealed that PGC-1α transcription interference contributes to HD neurodegeneration, linking the nuclear transcriptionopathy with the mitochondrial dysfunction. Subsequent work has strengthened this view, and further extended the role of PGC-1α within the CNS. Within the last year, studies of Parkinson’s disease, another involuntary movement disorder long associated with mitochondrial dysfunction, have shown that PGC-1α dysregulation is contributing to its pathogenesis. As PGC-1α is likely also important for aging, a process with considerable relevance to neuron function, translational studies aimed at developing therapies based upon the PGC-1α pathway as a high priority target are underway.</p>
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