Neuroprotective effects of L-carnitine in a transgenic animal model of Huntington's disease

被引：31

作者：

Vamos, Eniko ^{[1
]}

Voros, Krisztina ^{[1
]}

Laszlo Vecsei ^{[1
]}

Klivenyi, Peter ^{[1
]}

机构：

[1] Univ Szeged, Dept Neurol, Albert Szent Gyorgyi Clin Ctr, H-6725 Szeged, Hungary

来源：

BIOMEDICINE & PHARMACOTHERAPY | 2010年 / 64卷 / 04期

关键词：

Huntington's disease; L-carnitine; Antioxidant; MOUSE MODEL; NEURODEGENERATIVE DISEASES; INTRANUCLEAR INCLUSIONS; WIDESPREAD EXPRESSION; PROTEOMIC ANALYSIS; STRIATAL NEURONS; RAT-BRAIN; GENE; RECEPTOR; MICE;

D O I：

10.1016/j.biopha.2009.06.020

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：

Huntington's disease (HD) is an autosomal dominant inherited disorder, caused by an expanded polyglutamine region of a protein called huntingtin. The excitotoxicity, oxidative damage, mitochondrial dysfunction and altered membrane transport may have important roles in the pathogenesis of HD. L-carnitine plays a role in facilitating the mitochondrial transport of fatty acids, but it also protects the cells from oxidative damage. The aim of our study was to examine the effects of the intraperitoneally administered L-carnitine on the survival, behaviour and immunohistochemical changes in the N171-82Q transgenic mouse model of HD. Following L-carnitine administration the survival was improved by 14.9%. The motor activity was significantly ameliorated as compared with the control transgenic group. The L-carnitine treatment significantly reduced the neuronal loss and the number of neuronal intranuclear aggregates. These results suggest that L-carnitine may exert a neuroprotective effect by decreasing the oxidative damage. (C) 2009 Elsevier Masson SAS. All rights reserved.

引用

页码：282 / 286

页数：5

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