Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Center, Taoyuan 333, Taiwan.
College of Medicine, Chang Gung University, Taoyuan 333, Taiwan.
Cells. 2023 Jan 20;12(3):385. doi: 10.3390/cells12030385.
Alterations in lipid composition and disturbed lipoprotein metabolism are involved in the pathomechanism of Huntington's disease (HD). Here, we measured 112 lipoprotein subfractions and components in the plasma of 20 normal controls, 24 symptomatic (sympHD) and 9 presymptomatic (preHD) HD patients. Significant changes were found in 30 lipoprotein subfractions and components in all HD patients. Plasma levels of total cholesterol (CH), apolipoprotein (Apo)B, ApoB-particle number (PN), and components of low-density lipoprotein (LDL) were lower in preHD and sympHD patients. Components of LDL4, LDL5, LDL6 and high-density lipoprotein (HDL)4 demonstrated lower levels in preHD and sympHD patients compared with controls. Components in LDL3 displayed lower levels in sympHD compared with the controls, whereas components in very low-density lipoprotein (VLDL)5 were higher in sympHD patients compared to the controls. The levels of components in HDL4 and VLDL5 demonstrated correlation with the scores of motor assessment, independence scale or functional capacity of Unified Huntington's Disease Rating Scale. These findings indicate the potential of components of VLDL5, LDL3, LDL4, LDL5 and HDL4 to serve as the biomarkers for HD diagnosis and disease progression, and demonstrate substantial evidence of the involvement of lipids and apolipoproteins in HD pathogenesis.
脂质组成的改变和脂蛋白代谢的紊乱与亨廷顿病(HD)的发病机制有关。在这里,我们测量了 20 名正常对照者、24 名有症状(sympHD)和 9 名无症状(preHD)HD 患者的血浆中的 112 种脂蛋白亚组分和成分。所有 HD 患者中有 30 种脂蛋白亚组分和成分发生了显著变化。preHD 和 sympHD 患者的血浆总胆固醇(CH)、载脂蛋白(Apo)B、ApoB-颗粒数(PN)和低密度脂蛋白(LDL)的水平较低。与对照组相比,preHD 和 sympHD 患者的 LDL4、LDL5、LDL6 和高密度脂蛋白(HDL)4 的成分水平较低。与对照组相比,sympHD 患者的 LDL3 成分水平较低,而 VLDL5 的成分水平较高。HDL4 和 VLDL5 的成分水平与运动评估、独立性量表或统一亨廷顿病评定量表的功能能力评分呈正相关。这些发现表明 VLDL5、LDL3、LDL4、LDL5 和 HDL4 的成分有潜力作为 HD 诊断和疾病进展的生物标志物,并为脂质和载脂蛋白在 HD 发病机制中的作用提供了实质性证据。