trans-(−)-ε-Viniferin increases mitochondrial sirtuin 3 (SIRT3), activates AMP-activated protein kinase (AMPK), and protects cells in models of Huntington disease

J Fu, J Jin, RH Cichewicz, SA Hageman, TK Ellis… - Journal of Biological …, 2012 - jbc.org
Huntington disease (HD) is an inherited neurodegenerative disorder caused by an
abnormal polyglutamine expansion in the protein Huntingtin (Htt). Currently, no cure is
available for HD. The mechanisms by which mutant Htt causes neuronal dysfunction and
degeneration remain to be fully elucidated. Nevertheless, mitochondrial dysfunction has
been suggested as a key event mediating mutant Htt-induced neurotoxicity because
neurons are energy-demanding and particularly susceptible to energy deficits and oxidative …