Wild-type huntingtin protects neurons from excitotoxicity
نویسندگان
چکیده
منابع مشابه
Wild-type huntingtin protects from apoptosis upstream of caspase-3.
Expansion of a polyglutamine sequence in the N terminus of huntingtin is the gain-of-function event that causes Huntington's disease. This mutation affects primarily the medium-size spiny neurons of the striatum. Huntingtin is expressed in many neuronal and non-neuronal cell types, implying a more general function for the wild-type protein. Here we report that wild-type huntingtin acts by prote...
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Healthy neurons do not store glycogen while they do possess the machinery for the glycogen synthesis albeit at an inactive state. Neurons in the degenerating brain, however, are known to accumulate glycogen, although its significance was not well understood. Emerging reports present contrasting views on neuronal glycogen synthesis; a few reports demonstrate a neurotoxic effect of glycogen while...
متن کاملDysfunction of wild-type huntingtin in Huntington disease.
Huntingtin is the protein involved in Huntington disease (HD), an inherited neurodegenerative disease. Research activities have focused on the abnormal functions of mutant huntingtin. However, recent results indicate that wild-type huntingtin has important activities in brain neurons, suggesting that loss of these activities may play a role in HD.
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ژورنال
عنوان ژورنال: Journal of Neurochemistry
سال: 2006
ISSN: 0022-3042,1471-4159
DOI: 10.1111/j.1471-4159.2005.03605.x