The lack of non-steroid isoprenoids causes oxidative stress in patients with mevalonic aciduria

被引:19
作者
Celec, Peter [1 ,2 ]
Behuliak, Michal [1 ]
机构
[1] Comenius Univ, Fac Med, Inst Pathophysiol, Bratislava 81108, Slovakia
[2] Comenius Univ, Fac Nat Sci, Dept Mol Biol, Bratislava, Slovakia
关键词
D O I
10.1016/j.mehy.2007.08.030
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
Mevalonic aciduria belongs to a group of rare inherited metabolic disorders related to cholesterol biosynthesis. The pathogenesis of mevalonic aciduria is not clear, although the cause is known -a genetic defect leading to a deficiency in mevalonate kinase activity. The tack of non-steroid isoprenoids in patients with mevalonic aciduria might cause oxidative stress due to a decreased production of endogenous antioxidants including ubiquinone 10, selenoproteins and glutathione peroxidase. The Moosmann-Behl hypothesis of statin-induced muscular and neuronal damage mediated by oxidative stress might explain at least partially the pathogenesis of mevalonic aciduria. Studies focusing on the role of oxidative stress in patients suffering from disorders in cholesterol biosynthesis are needed to support adjuvant antioxidative treatment. (C) 2007 Elsevier Ltd. All rights reserved.
引用
收藏
页码:938 / 940
页数:3
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