| Interacting proteins: P55212 and P42858 |
Pubmed |
SVM Score :0.0 |
| Additionally , we define htt itself as an important caspase substrate by generating a site directed htt mutant that is resistant to caspase 3 cleavage at positions 513 and 530 and to caspase 6 cleavage at position 586 . ^^^ |
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| Interacting proteins: P55212 and P42858 |
Pubmed |
SVM Score :0.0 |
| We also identify caspase 6 and caspase 3 cleavage sites in huntingtin and demonstrate that neuronal and non neuronal cells expressing a caspase resistant huntingtin with an expanded polyglutamine tract are less susceptible to apoptosis and aggregate formation . ^^^ |
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| Interacting proteins: P55212 and P42858 |
Pubmed |
SVM Score :0.0 |
| To determine whether caspase cleavage of htt is a key event in the neuronal dysfunction and selective neurodegeneration in HD , we generated YAC mice expressing caspase 3 and caspase 6 resistant mutant htt . ^^^ These results are consistent with proteolysis of htt at the caspase 6 cleavage site being an important event in mediating neuronal dysfunction and neurodegeneration and highlight the significant role of htt proteolysis and excitotoxicity in HD . . ^^^ Mice expressing mutant htt , resistant to cleavage by caspase 6 but not caspase 3 , maintain normal neuronal function and do not develop striatal neurodegeneration . ^^^ Furthermore , caspase 6 resistant mutant htt mice are protected against neurotoxicity induced by multiple stressors including NMDA , quinolinic acid ( QA ) , and staurosporine . ^^^ Cleavage at the caspase 6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin . ^^^ |
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