Article
Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.
Proceedings of the National Academy of Sciences of the United States of America - 23 Oct 2001
Kim Y J, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel K B, Qin Z H, Aronin N, DiFiglia M
Abstract excerpt
The Huntington's disease (HD) mutation is a polyglutamine expansion in the N-terminal region of huntingtin (N-htt). How neurons die in HD is unclear. Mutant N-htt aggregates in neurons in the HD brain; expression of mutant N-htt in vitro causes cell death. Other in vitro studies show that proteolysis by caspase 3 could be important in regulating mutant N-htt function, but there has been no direct evidence for...
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