Article
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease.
Nature medicine - 1 May 2014
Wang Nan, Gray Michelle, Lu Xiao-Hong, Cantle Jeffrey P, Holley Sandra M, Greiner Erin, Gu Xiaofeng, Shirasaki Dyna, Cepeda Carlos, Li Yuqing, Dong Hongwei, Levine Michael S, Yang X William
Abstract excerpt
Huntington's disease (HD) is a fatal dominantly inherited neurodegenerative disorder caused by a CAG repeat expansion leading to an elongated polyglutamine stretch in huntingtin. Mutant huntingtin (mHTT) is ubiquitously expressed in all cells but elicits selective cortical and striatal neurodegeneration in HD. The mechanistic basis for such selective neuronal vulnerability remains unclear. A necessary step toward...
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