PGC-1α, a New Therapeutic Target in Huntington's Disease?
Open accessAbstract
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.
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- Authors
- McGill, J. K., & Beal, M. F.
- Affiliations
- Weill Cornell Medical Center (US)
- Corresponding author
- Beal, M. Flint
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2006-11
- Volume
- 127
- Issue
- 3
- Pages
- 465-468
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2172197220
- Cited by
- 131
- References
- 20
- Citations by year
- 2025: 2 · 2023: 2 · 2022: 5 · 2021: 5 · 2020: 3 · 2019: 4 · 2018: 7 · 2017: 5 · 2016: 6 · 2015: 6 · 2014: 9 · 2013: 8 · 2012: 11
- FWCI
- 4.28
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- www.elsevier.com/legal/tdmrep-license (tdm)
- www.elsevier.com/open-access/userlicense/1.0/ (vor, 2450 d)
- doi.org/10.15223/policy-017 (stm-asf)
- doi.org/10.15223/policy-037 (stm-asf)
- doi.org/10.15223/policy-012 (stm-asf)
- doi.org/10.15223/policy-029 (stm-asf)
- doi.org/10.15223/policy-004 (stm-asf)
- Locations
- Cell (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Keywords
- PGC-1α, Huntington's disease, mitochondrial biogenesis, mitochondrial respiration, energy metabolism, neurodegeneration, transcriptional coactivator, therapeutic target
- MeSH terms
- Huntingtin Protein, Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha, Animals, Energy Metabolism, Heat-Shock Proteins, Humans, Huntington Disease, Nerve Tissue Proteins, Nuclear Proteins, Transcription Factors, Trans-Activators, Down-Regulation, Neurodegenerative Diseases, Mice
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