Methylmalonic acid -: an endogenous toxin?

被引：52

作者：

Kölker, S ^{[1
]}

Okun, JG ^{[1
]}

机构：

[1] Heidelberg Univ, Childrens Hosp, Dept Gen Pediat, Div Inborn Metab Dis, D-69120 Heidelberg, Germany

来源：

CELLULAR AND MOLECULAR LIFE SCIENCES | 2005年 / 62卷 / 06期

关键词：

methylmalonic aciduria; organic acids; metabolic stroke; excitotoxicity; energy metabolism; renal failure;

D O I：

10.1007/s00018-005-4463-2

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Methylmalonic acid was previously considered as major neurotoxin in methylmalonic acidurias. In contrast, recent studies support the notion that other metabolites deriving from propionyl-coenzyme A, inducing synergistic inhibition of mitochondrial energy metabolism, are more important than methylmalonic acid to understand the neuropathogenesis of this disease. However, it is not yet known whether methylmalonic acid is involved in the induction of other organ manifestations in this disease, such as chronic renal failure.

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页码：621 / 624

页数：4

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