Neuroprotective effects of L-carnitine in a transgenic animal model of Huntington's disease.
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.
Vamos
Eniko
E
Department of Neurology, Albert Szent-Gyorgyi Clinical Centre, University of Szeged, Semmelweis str u. 6., 6725 Szeged, Hungary.
Voros
Krisztina
K
Vecsei
Laszlo
L
Klivenyi
Peter
P
D000818:Animals; D001522:Behavior, Animal / Q000187:drug effects; D002331:Carnitine / Q000494:pharmacology*; D004195:Disease Models, Animal; D006816:Huntington Disease / Q000188:drug therapy* / Q000503:physiopathology; D007274:Injections, Intraperitoneal; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009043:Motor Activity / Q000187:drug effects; D009474:Neurons / Q000187:drug effects / Q000378:metabolism; D018696:Neuroprotective Agents / Q000494:pharmacology*; D018384:Oxidative Stress / Q000187:drug effects*; D013534:Survival
D018696:Neuroprotective Agents; D002331:Carnitine