Emerging roles for cholesterol in Huntington's disease

Abstract

Recent findings suggest that alterations in cholesterol homeostasis might be associated with Huntington's disease (HD). Although alterations in cholesterol content have been detected in cell models and several rodent models of HD, it is currently unclear what the potential mechanisms underlying cholesterol perturbations are. Furthermore, it remains to be determined whether these observed changes in cholesterol homeostasis are involved in the pathogenesis of HD or occur as a secondary event. In this review, we provide an overview of current studies that have begun to address these issues and discuss recent findings suggesting that normal huntingtin protein might participate in regulating cholesterol biosynthesis. A better understanding of how alterations in cholesterol levels contribute to the pathophysiology of HD is likely to have significant therapeutic implications for the treatment of this debilitating neurodegenerative disorder.