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Letter

Nature Genetics 37, 198–204 (1 February 2005) | doi:10.1038/ng1503

Polyglutamine expansion of huntingtin impairs its nuclear export

Jonathan Cornett , Fengli Cao , Chuan-En Wang , Christopher A Ross , Gillian P Bates , Shi-Hua Li & Xiao-Jiang Li

Proteins with polyglutamine (polyQ) expansions accumulate in the nucleus and affect gene expression. The mechanism by which mutant huntingtin (htt) accumulates intranuclearly is not known; wild-type htt, a 350-kDa protein of unknown function, is normally found in the cytoplasm.