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Article

PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function.

Science translational medicine - 11 Jul 2012

Tsunemi Taiji, Ashe Travis D, Morrison Bradley E, Soriano Kathryn R, Au Jonathan, Roque Ruben A Vázquez, Lazarowski Eduardo R, Damian Vincent A, Masliah Eliezer, La Spada Albert R

Abstract excerpt

Huntington's disease (HD) is caused by CAG repeat expansions in the huntingtin (htt) gene, yielding proteins containing polyglutamine repeats that become misfolded and resist degradation. Previous studies demonstrated that mutant htt interferes with transcriptional programs coordinated by the peroxisome proliferator-activated receptor γ (PPARγ) coactivator 1α (PGC-1α), a regulator of mitochondrial biogenesis and...

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Share to Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Nerve Degeneration

Share to Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha

Share to Protein Structure, Quaternary

Share to Reactive Oxygen Species

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Share to Trinucleotide Repeat Expansion

Source

Open in PubMed Open full text

Identifiers

PMID: 22786682
DOI: 10.1126/scitranslmed.3003799
PMCID: PMC4096245

Genes

PGC
TFEB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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