Article
Mutant huntingtin can paradoxically protect neurons from death.
Cell death and differentiation - 1 Mar 2008
Zuchner T, Brundin P
Abstract excerpt
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a mutation in the gene huntingtin and characterized by motor, cognitive and psychiatric symptoms. Huntingtin contains a CAG repeat in exon 1. An expansion of this CAG repeat above 35 results in misfolding of Huntingtin, giving rise to protein aggregates and neuronal cell death. There are several transgenic HD mouse models that...
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