Article
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease.
Science (New York, N.Y.) - 20 Jul 2001
Zuccato C, Ciammola A, Rigamonti D, Leavitt B R, Goffredo D, Conti L, MacDonald M E, Friedlander R M, Silani V, Hayden M R, Timmusk T, Sipione S, Cattaneo E
Abstract excerpt
Huntingtin is a 350-kilodalton protein of unknown function that is mutated in Huntington's disease (HD), a neurodegenerative disorder. The mutant protein is presumed to acquire a toxic gain of function that is detrimental to striatal neurons in the brain. However, loss of a beneficial activity of wild-type huntingtin may also cause the death of striatal neurons. Here we demonstrate that wild-type huntingtin...
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