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Transcription meets metabolism in neurodegeneration

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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.

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Figure 1: PGC-1α regulation of mitochondria is impaired in Huntington disease.

Kim Caesar

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Authors and Affiliations

  1. 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, 21287, Maryland, USA

    Christopher A Ross

  2. Departments of Psychiatry and Human Behavior, Biological Chemistry, and Neurobiology and Behavior, Gillespie 2121, University of California, Irvine, 92697-4260, California, USA

    Leslie Michels Thompson

Authors
  1. Christopher A Ross
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  2. Leslie Michels Thompson
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Ross, C., Thompson, L. Transcription meets metabolism in neurodegeneration. Nat Med 12, 1239–1241 (2006). https://doi.org/10.1038/nm1106-1239

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