Neurodegenerative disease: Preventing 'SIRTain' death by mutant huntingtin.

摘要

Huntington's disease is an inherited neuro-degenerative disease caused by a glutamine repeat expansion in huntingtin (HTT) protein that results in HTT accumulation. Studies have shown that calorie restriction ameliorates the pathogenesis of the disease in mice, but the role of the NAD-dependent protein deacetylase sirtuin 1 (SIRT1), which is one of the mediators of the beneficial roles of calorie restriction in Huntingtons disease, was unclear. Two studies in Nature Medicine reveal a neuroprotective role for this enzyme and, thus, potential new therapeutic opportunities for treating the disease.