Nature Medicine
- 12, 1239 - 1241 (2006)
doi:10.1038/nm1106-1239
Transcription meets metabolism in neurodegenerationChristopher A Ross1 & Leslie Michels Thompson21
Christopher A. Ross is in the Division of Neurobiology of the Department of Psychiatry and the Departments of Neurology and Neuroscience, Johns Hopkins University School of Medicine, 600 North Wolfe Street, Baltimore, Maryland 21287, USA. caross@jhu.edu
2
Leslie Michels Thompson is in the Departments of Psychiatry and Human Behavior, Biological Chemistry, and Neurobiology and Behavior, Gillespie 2121, University of California, Irvine, California 92697-4260, USA. kanjiA molecular basis for the metabolic abnormalities observed in Huntington disease is emerging. Transcription of a key mitochondrial regulator, PGC-1 , 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.
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