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 586 of mutant huntingtin prevents the development of behavioural, motor and neuropathological features in a mouse model...

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Veröffentlicht in:	Human molecular genetics 2008-08, Vol.17 (15), p.2390-2404
Hauptverfasser:	Warby, Simon C., Doty, Crystal N., Graham, Rona K., Carroll, Jeffrey B., Yang, Yu-Zhou, Singaraja, Roshni R., Overall, Christopher M., Hayden, Michael R.
Format:	Artikel
Sprache:	eng
Schlagworte:	Active Transport, Cell Nucleus Ageing, cell death Amino Acid Sequence Animals Biological and medical sciences Caspase 6 - metabolism Cell Line Cell Nucleus - enzymology Cell Nucleus - metabolism Cell physiology Cercopithecus aethiops COS Cells Cytoplasm - enzymology Enzyme Activation Fundamental and applied biological sciences. Psychology Genetics of eukaryotes. Biological and molecular evolution Humans Huntingtin Protein Mice Mice, Transgenic Molecular and cellular biology Molecular Sequence Data Mutation Nerve Tissue Proteins - genetics Nerve Tissue Proteins - metabolism Nuclear Proteins - genetics Nuclear Proteins - metabolism Protein Structure, Tertiary
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