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Article

Truncated peroxisome proliferator-activated receptor-γ coactivator 1α splice variant is severely altered in Huntington's disease.

Neuro-degenerative diseases - 1 Jan 2011

Johri Ashu, Starkov Anatoly A, Chandra Abhishek, Hennessey Thomas, Sharma Abhijeet, Orobello Sara, Squitieri Ferdinando, Yang Lichuan, Beal M Flint

Abstract excerpt

BACKGROUND: Reduced peroxisome proliferator-activated receptor-γ coactivator 1α (PGC1α) gene expression has been observed in striatal cell lines, transgenic mouse models of Huntington's disease (HD), and brain tissue from HD patients. As this protein is a key transcription regulator of the expression of many mitochondrial proteins, these observations strongly support the role of aberrant mitochondrial function in...

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Identifiers

PMID: 21757867
DOI: 10.1159/000327910
PMCID: PMC3186722

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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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