Benefit of a combined treatment with trientine and ascorbate in familial amyotrophic lateral sclerosis model mice

被引：65

作者：

Nagano, S ^{[1
]}

Ogawa, Y ^{[1
]}

Yanagihara, T ^{[1
]}

Sakoda, S ^{[1
]}

机构：

[1] Osaka Univ, Grad Sch Med, Dept Neurol D4, Osaka, Japan

来源：

NEUROSCIENCE LETTERS | 1999年 / 265卷 / 03期

关键词：

familial amyotrophic lateral sclerosis; copper-zinc superoxide dismutase; copper; transgenic mice; trientine; ascorbate;

D O I：

10.1016/S0304-3940(99)00227-X

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

We previously reported that the common toxic gain-of-function in various mutant copper-zinc superoxide dismutases (SOD1) seen in patients with familial amyotrophic lateral sclerosis (ALS) was an abnormal copper release from the enzyme protein. In this study, trientine and ascorbate, known to have a beneficial effect in an animal model of Wilson disease, were administered to transgenic mice overexpressing a mutated human SOD1 (G93A). The onset of neurological signs in the treated group was significantly delayed compared with that in the control group, and the time to reach total paralysis in the treated group was delayed as well. Since the agents used in this study cause low toxicity in animals and humans, this treatment may be a good candidate for clinical application. (C) 1999 Elsevier Science Ireland Ltd. All rights reserved.

引用

页码：159 / 162

页数：4

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