In vivo characterization of caspase resistant huntingtin : insights into the pathogenic mechanism of Huntington disease
Proteolytic cleavage of htt is regarded as a critical event in the pathogenesis of HD. Expression of htt fragments containing an expanded polyglutamine repeat are toxic in vitro and in vivo, and accumulation of N-terminal truncated products of htt are observed in human and mouse HD brain. Notably...
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| Language: | English |
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2010
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| Online Access: | http://hdl.handle.net/2429/18441 |