Article
Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity.
Nature genetics - 1 Aug 2000
Li H, Li S H, Johnston H, Shelbourne P F, Li X J
Abstract excerpt
Huntington disease (HD) is caused by expansion of a glutamine repeat in the amino-terminal region of huntingtin. Despite its widespread expression, mutant huntingtin induces selective neuronal loss in striatal neurons. Here we report that, in mutant mice expressing HD repeats, the production and aggregation of N-terminal huntingtin fragments preferentially occur in HD-affected neurons and their processes and...
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