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Article

Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice.

The Journal of biological chemistry - 2 Apr 2010

Bradford Jennifer, Shin Ji-Yeon, Roberts Meredith, Wang Chuan-En, Sheng Guoqing, Li Shihua, Li Xiao-Jiang

Abstract excerpt

Huntington disease (HD) is caused by an expansion of the polyglutamine (polyQ) repeat (>37Q) in huntingtin (htt), and age of onset is inversely correlated with the length of the polyQ repeat. Mutant htt with expanded polyQ is ubiquitously expressed in various types of cells, including glia, but causes selective neurodegeneration. Our recent study demonstrated that expression of the N-terminal mutant htt with a...

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Identifiers

PMID: 20145253
DOI: 10.1074/jbc.M109.083287
PMCID: PMC2856273

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