A molecular basis for the metabolic abnormalities observed in Huntington disease is emerging. Transcription of a key mitochondrial regulator, PGC-1α, is dysregulated by mutant huntingtin, leading to oxidative stress and excitotoxicity. The findings dovetail with previous work implicating aberrant transcription in Huntington disease, and have implications for related conditions such as Parkinson disease.
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)