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Nature Cell Biology 7, 650 (1 July 2005) | doi:10.1038/ncb0705-650

Huntingtin and the vicious circle

Jack Horne

Huntington's disease is a neurodegenerative disorder, caused by mutations in the huntingtin (htt) protein that result in expanded amino-terminal polyglutamine tracts. Recent data from genetic studies and transgenic mouse models suggest that N-terminal fragments of the expanded protein — released by proteolytic cleavage — may be the toxic species responsible for neuronal death and disease pathology.