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Transgenic Mouse Model Expressing the Caspase 6 Fragment of Mutant Huntingtin

4 Jan 2012

Abstract excerpt

Huntington's disease (HD) is caused by a polyglutamine expansion in the Huntingtin (Htt) protein. Proteolytic cleavage of Htt into toxic N-terminal fragments is believed to be a key aspect of pathogenesis. The best characterized putative cleavage event is at amino acid 586, hypothesized to be mediated by caspase 6. A corollary of the caspase 6 cleavage hypothesis is that the caspase 6 fragment should be a toxic...

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PMID: 22219281
DOI: 10.1523/jneurosci.1305-11.2012

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