The impairment of cholesterol metabolism in Huntington disease

Article ID	Journal	Published Year	Pages	File Type
8301951	Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids	2015	11 Pages	PDF

Abstract

HTT seems to play a regulatory role in lipid metabolism. The impairment of the cholesterol metabolism was found to be proportional to the CAG repeat length and to the load of mutant HTT. A dysregulation on PGC1Î± and mitochondria dysfunction may be involved in an overall reduction of acetyl-CoA and ATP synthesis, contributing to the cerebral and whole body cholesterol impairment. This article is part of a Special Issue entitled Brain Lipids.

Keywords

PPARγ SRE PLP htt VLDL NPC LXR CYP51 DHCR24 SREBP PGC1α ACAT LDL-R CYP27A1 CAV1 TCA HDL ABC sterol responsive element binding protein YAC HMGCoAR CYP7A1 Neural stem CYP46A1 HMGCoA SCAP MBP LRP 24-dehydrocholesterol reductase 24S-hydroxycholesterol 27-Hydroxycholesterol acyl-CoA:cholesterol acyltransferase BDNF HMGCoA reductase LDL-receptor Apoe apolipoprotein E BACHD sterol 27-hydroxylase tricarboxylic acid MRI Alzheimer disease Huntington disease Insig Biomarker Magnetic resonance imaging Neurodegeneration Striatal ATP-binding cassette transporter CNS central nervous system endoplasmic reticulum Mass spectrometry Brain-derived neurotrophic factor low density lipoproteins very low density lipoprotein LDL high density lipoproteins Lipid CSF Cerebrospinal fluid Metabolomics Multiple sclerosis Niemann–Pick type C Huntingtin srebp cleavage-activating protein Myelin basic protein Insulin Induced Gene caveolin-1 liver X receptor Yeast artificial chromosome Cholesterol 24-hydroxylase cholesterol 7α-hydroxylase