Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin
RK Graham, Y Deng, EJ Slow, B Haigh, N Bissada… - Cell, 2006 - cell.com
Cleavage of huntingtin (htt) has been characterized in vitro, and accumulation of caspase
cleavage fragments represents an early pathological change in brains of Huntington's …
cleavage fragments represents an early pathological change in brains of Huntington's …
Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo
Caspase cleavage of huntingtin (htt) and nuclear htt accumulation represent early
neuropathological changes in brains of patients with Huntington's disease (HD). However …
neuropathological changes in brains of patients with Huntington's disease (HD). However …
Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus
Proteolysis of mutant huntingtin is crucial to the development of Huntington disease (HD).
Specifically preventing proteolysis at the capase-6 (C6) consensus sequence at amino acid …
Specifically preventing proteolysis at the capase-6 (C6) consensus sequence at amino acid …
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease
CL Wellington, LM Ellerby, CA Gutekunst… - Journal of …, 2002 - Soc Neuroscience
Huntington's disease (HD) results from polyglutamine expansion in huntingtin (htt), a protein
with several consensus caspase cleavage sites. Despite the identification of htt fragments in …
with several consensus caspase cleavage sites. Despite the identification of htt fragments in …
Huntington disease: new insights on the role of huntingtin cleavage
CL Wellington, BR Leavitt, MR Hayden - Advances in Research on …, 2000 - Springer
Huntington Disease (HD) results from polyglutamine expansion within the N-terminus of
huntingtin. We have produced yeast artificial chromosome (YAC) transgenic mice …
huntingtin. We have produced yeast artificial chromosome (YAC) transgenic mice …
Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and …
YJ Kim, Y Yi, E Sapp, Y Wang… - Proceedings of the …, 2001 - National Acad Sciences
The Huntington's disease (HD) mutation is a polyglutamine expansion in the N-terminal
region of huntingtin (N-htt). How neurons die in HD is unclear. Mutant N-htt aggregates in …
region of huntingtin (N-htt). How neurons die in HD is unclear. Mutant N-htt aggregates in …
Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease
E Hermel, J Gafni, SS Propp, BR Leavitt… - Cell Death & …, 2004 - nature.com
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative
disorder resulting in selective neuronal loss and dysfunction in the striatum and cortex. The …
disorder resulting in selective neuronal loss and dysfunction in the striatum and cortex. The …
Wild‐type huntingtin protects neurons from excitotoxicity
BR Leavitt, JM van Raamsdonk… - Journal of …, 2006 - Wiley Online Library
Huntingtin is a caspase substrate, and loss of normal huntingtin function resulting from
caspase‐mediated proteolysis may play a role in the pathogenesis of Huntington disease …
caspase‐mediated proteolysis may play a role in the pathogenesis of Huntington disease …
Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
CL Wellington, L Ellerby, J Savill, S Roy… - Journal of Biological …, 2000 - ASBMB
Huntington's disease is a neurodegenerative disorder caused by CAG expansion that
results in expansion of a polyglutamine tract at the extreme N terminus of huntingtin (htt). htt …
results in expansion of a polyglutamine tract at the extreme N terminus of huntingtin (htt). htt …
Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing
D Rigamonti, S Sipione, D Goffredo, C Zuccato… - Journal of Biological …, 2001 - ASBMB
Huntington's Disease is an inherited neurodegenerative disease that affects the medium
spiny neurons in the striatum. The disease is caused by the expansion of a polyglutamine …
spiny neurons in the striatum. The disease is caused by the expansion of a polyglutamine …
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