Article
Mutant protein in Huntington disease is resistant to proteolysis in affected brain.
Nature genetics - 1 Nov 2001
Dyer R B, McMurray C T
Abstract excerpt
The cause of Huntington disease pathophysiology is unknown, but a major hypothesis suggests that toxicity arises from the cleavage and accumulation of amino-terminal fragments containing an expanded polyglutamine region. In evaluating huntingtin protein (HD) from human brain, transgenic animals and cells, we observed, unexpectedly, that mutant HD is more resistant to proteolysis than normal HD. The N-terminal...
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