Article
Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease.
Human molecular genetics - 1 Mar 2012
Johri Ashu, Calingasan Noel Y, Hennessey Thomas M, Sharma Abhijeet, Yang Lichuan, Wille Elizabeth, Chandra Abhishek, Beal M Flint
Abstract excerpt
There is substantial evidence that impairment of peroxisome proliferator-activated receptor (PPAR)-γ-coactivator 1α (PGC-1α) levels and activity play an important role in Huntington's disease (HD) pathogenesis. We tested whether pharmacologic treatment with the pan-PPAR agonist bezafibrate would correct a deficiency of PGC-1α and exert beneficial effects in a transgenic mouse model of HD. We found that...
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