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Dysfunction of wild-type huntingtin in Huntington disease
1Department of Pharmacological Sciences and Center for Excellence in Neurodegenerative Diseases, University of Milan, 20133 Milan, Italy.
Abstract:
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.