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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References
Cui, L. et al. Cell 127, 59–69 (2006).
Weydt, P. et al. Cell Metab. published online 19 October 2006 (doi: 10.1016/jcmet.2006.10.004).
St-Pierre, J. et al. Cell 127, 397–408 (2006).
Beal, M.F. Ann. Neurol. 58, 495–505 (2005).
Ross, C.A. Neuron 35, 819–822 (2002).
Sugars, K.L. & Rubinsztein, D.C. Trends Genet. 19, 233–238 (2003).
Handschin, C. & Spiegelman, B.M. Endocr. Rev., published online 3 October 2006 (doi:10.1210/er.2006-0037).
Lin, J. et al. Cell 119, 121–135 (2004).
Mantamadiotis, T. et al. Nat. Genet. 31, 47–54 (2002).
Bae, B.I. et al. Neuron 47, 29–41 (2005).
Cattaneo, E., Zuccato, C. & Tartari, M. Nat. Rev. Neurosci. 6, 919–930 (2005).
Nonomura, T. et al. Int. J. Exp. Diabetes Res. 2, 201–209 (2001).
Djousse, L. et al. Neurology 59, 1325–1330 (2002).
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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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DOI: https://doi.org/10.1038/nm1106-1239
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