CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington’s disease

Boussicault, Lydie; Alves, Sandro; Lamazière, Antonin; Planques, Anabelle; Heck, Nicolas; Moumné, Lara; Després, Gaëtan; Bolte, Susanne; Hu, Amélie; Pagès, Christiane; Galvan, Laurie; Piguet, Françoise; Aubourg, Patrick; Cartier, Nathalie; Caboche, Jocelyne; Bétuing, Sandrine

doi:10.1093/brain/awv384

Public

CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington’s disease

Published in Brain • Jan 29, 2016

Authors:

Lydie Boussicault

Sandro Alves

Antonin Lamazière

Abstract

Huntington's disease is an autosomal dominant neurodegenerative disease caused by abnormal polyglutamine expansion in huntingtin (Exp-HTT) leading to degeneration of striatal neurons. Altered brain cholesterol homeostasis has been implicated in Huntington's disease, with increased accumulation of ch...

Subject

Huntingtin

Huntington's disease

Striatum

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