Cited by 131Genetic Neurodegenerative DiseasesMitochondrial Function and PathologyParkinson's Disease Mechanisms and Treatments

PGC-1α, a New Therapeutic Target in Huntington's Disease?

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McGill, Jetta K., Beal, M. Flint·2006·Cell

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.

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Crossref · 10.1016/j.cell.2006.10.023

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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
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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