辅酶Q2表达的降低与多系统萎缩症大脑中ATP水平的降低有关。
Reductions in COQ2 Expression Relate to Reduced ATP Levels in Multiple System Atrophy Brain.
作者信息
Hsiao Jen-Hsiang T, Purushothuman Sivaraman, Jensen Poul H, Halliday Glenda M, Kim Woojin Scott
机构信息
Brain and Mind Centre and Central Clinical School, The University of Sydney, Sydney, NSW, Australia.
School of Medical Sciences, University of New South Wales & Neuroscience Research Australia, Randwick, NSW, Australia.
出版信息
Front Neurosci. 2019 Nov 1;13:1187. doi: 10.3389/fnins.2019.01187. eCollection 2019.
Multiple system atrophy (MSA) is a progressive neurodegenerative disease clinically characterized by parkinsonism and cerebellar ataxia, and pathologically by oligodendrocyte α-synuclein inclusions. Genetic variants of COQ2 are associated with an increased risk for MSA in certain populations. Also, deficits in the level of coenzyme Q and its biosynthetic enzymes are associated with MSA. Here, we measured ATP levels and expression of biosynthetic enzymes for coenzyme Q, including COQ2, in multiple regions of MSA and control brains. We found a reduction in ATP levels in disease-affected regions of MSA brain that associated with reduced expression of COQ2 and COQ7, supporting the concept that abnormalities in the biosynthesis of coenzyme Q play an important role in the pathogenesis of MSA.
多系统萎缩（MSA）是一种进行性神经退行性疾病，临床特征为帕金森综合征和小脑共济失调，病理特征为少突胶质细胞α-突触核蛋白包涵体。在某些人群中，COQ2的基因变异与MSA风险增加有关。此外，辅酶Q及其生物合成酶水平的缺陷也与MSA有关。在此，我们测量了MSA患者和对照者大脑多个区域的ATP水平以及辅酶Q生物合成酶（包括COQ2）的表达。我们发现MSA患者大脑中受疾病影响区域的ATP水平降低，这与COQ2和COQ7表达减少有关，支持了辅酶Q生物合成异常在MSA发病机制中起重要作用的观点。
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