Article
Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus.
Human molecular genetics - 1 Aug 2008
Warby Simon C, Doty Crystal N, Graham Rona K, Carroll Jeffrey B, Yang Yu-Zhou, Singaraja Roshni R, Overall Christopher M, Hayden Michael R
Abstract excerpt
Proteolysis of mutant huntingtin is crucial to the development of Huntington disease (HD). Specifically preventing proteolysis at the capase-6 (C6) consensus sequence at amino acid 586 of mutant huntingtin prevents the development of behavioural, motor and neuropathological features in a mouse model of HD. However, the mechanism underlying the selective toxicity of the 586 amino acid cleavage event is currently...
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