Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.

@article{Weydt2006ThermoregulatoryAM,
  title={Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.},
  author={Patrick Weydt and Victor V. Pineda and Anne E Torrence and Randell T. Libby and Terrence Forrest Satterfield and Eduardo Rodolfo Lazarowski and Merle L. Gilbert and Gregory J Morton and Theodor K. Bammler and Andrew D. Strand and Libin Cui and Richard P. Beyer and Courtney N Easley and Annette C. Smith and Dimitri Krainc and Serge Luquet and Ian Sweet and Michael J. Schwartz and Albert R La Spada},
  journal={Cell metabolism},
  year={2006},
  volume={4 5},
  pages={
          349-62
        }
}
Huntington's disease (HD) is a fatal, dominantly inherited disorder caused by polyglutamine repeat expansion in the huntingtin (htt) gene. Here, we observe that HD mice develop hypothermia associated with impaired activation of brown adipose tissue (BAT). Although sympathetic stimulation of PPARgamma coactivator 1alpha (PGC-1alpha) was intact in BAT of HD mice, uncoupling protein 1 (UCP-1) induction was blunted. In cultured cells, expression of mutant htt suppressed UCP-1 promoter activity… CONTINUE READING
BETA

Citations

Publications citing this paper.
SHOWING 1-10 OF 273 CITATIONS

Similar Papers

Loading similar papers…