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摘要:
Coenzyme Q10(CoQ10)is an essential cofactor in the mitochondrial respiratory pathway and also functions as a lipid-soluble antioxidant.CoQ10 deficiency has been implicated in many clinical disorders and aging.Primary CoQ10 deficiency is a group of recessively inherited diseases caused by mutations in any gene involved in the CoQ10 biosynthesis pathway.Although primary CoQ10 deficiency is rare,its diagnosis is important because it is potentially treatable with exogenous CoQ10.Multiple system atrophy(MSA)was recently shown to be linked to mutations in the COQ2 gene,one of the genes involved in the CoQ10 biosynthesis pathway.MSA is relatively common in adult-onset neurodegenerative diseases characterized by Parkinsonism,cerebellar ataxia and autonomic failures.Because COQ2 mutations are associated with an increased risk of MSA,oral CoQ10 supplementation may be beneficial for MSA,as for other primary CoQ10 deficiencies.Statins are 3-hydroxy-3-methylglutaryl coenzyme A inhibitors that inhibit the biosynthesis of cholesterol,as well as the synthesis of mevalonate,a critical intermediate in cholesterol synthesis.Statin therapy has been associated with a variety of muscle complaints from myalgia to rhabdomyolysis.Statin treatment carries a potential risk of CoQ10 deficiency,although no definite evidence has implicated CQ10 deficiency as the cause of statinrelated myopathy.
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篇名 Coenzyme Q10 in neurodegenerative disorders:Potential benefit of CoQ10 supplementation for multiple system atrophy
来源期刊 世界神经病学杂志 学科 医学
关键词 Primary COENZYME Q10 deficiency Multiple system ATROPHY CEREBELLAR ATAXIA COQ2 gene STATIN COENZYME Q10 supplementation Reduced COENZYME Q10
年,卷(期) 2014,(1) 所属期刊栏目
研究方向 页码范围 1-6
页数 6页 分类号 R
字数 语种
DOI
五维指标
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研究主题发展历程
节点文献
Primary
COENZYME
Q10
deficiency
Multiple
system
ATROPHY
CEREBELLAR
ATAXIA
COQ2
gene
STATIN
COENZYME
Q10
supplementation
Reduced
COENZYME
Q10
研究起点
研究来源
研究分支
研究去脉
引文网络交叉学科
相关学者/机构
期刊影响力
世界神经病学杂志
不定期
2218-6212
北京市朝阳区东四环中路62号楼远洋国际中
出版文献量(篇)
48
总下载数(次)
0
总被引数(次)
0
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