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. 2016 Jan 29;139(3):953–970. doi: 10.1093/brain/awv384

Figure 7.

Figure 7

Regulation of cholesterol homeostasis by CYP46A1 is neuroprotective in Huntington’s disease. Left : Cholesterol homeostasis is dramatically dysregulated in Huntington’s disease (HD). Despite a decrease of lanosterol and 7-lathosterol, cholesterol is slightly increased, due to down regulation of CYP46A1 expression. Right : Restoration of CYP46A1 expression in Huntington’s disease increases cholesterol catabolism, thereby decreasing cholesterol content. It also increases levels of lanosterol and desmosterol, which protect striatal neurons from Exp-HTT-mediated toxicity. CYP46A1 displays a potent neuroprotective effect in Huntington’s disease likely by increasing cholesterol turnover and Bloch pathway activity.