Is Amyotrophic Lateral Sclerosis a Mitochondrial Channelopathy?

被引：9

作者：

Le Verche, Virginia ^{[1
,2
]}

Przedborski, Serge ^{[1
,2
]}

机构：

[1] Columbia Univ, Dept Neurol Pathol & Cell Biol, New York, NY 10032 USA

[2] Columbia Univ, Ctr Motor Neuron Biol & Dis, New York, NY 10032 USA

来源：

NEURON | 2010年 / 67卷 / 04期

关键词：

MUTANT SOD1; TOXICITY; MICE;

D O I：

10.1016/j.neuron.2010.08.010

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

SOD1 is a cause of the fatal, paralytic disorder ALS. Although mechanisms underlying mutant SOD1 neurotoxicity remain uncertain, this protein associates with mitochondria. In this issue of Neuron, Israelson et al. show that mutant SOD1 binds and inhibits the mitochondria! channel VDAC1. This finding sheds light onto possible molecular links between mutant SOD1, mitochondrial dysfunction, and spinal motor neuron degeneration in inherited ALS.

引用

页码：523 / 524

页数：2

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