Journal
NEUROBIOLOGY OF DISEASE
Volume 20, Issue 2, Pages 267-274Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2005.02.013
Keywords
Huntington's disease; perinuclear site; Htt; cleavage; caspase
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Funding
- NIDA NIH HHS [DA-00074] Funding Source: Medline
- NIMH NIH HHS [MH-69853, MH-18501] Funding Source: Medline
- NINDS NIH HHS [NS-34172/NS-16375] Funding Source: Medline
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Accumulation of mutant Huntingtin (Htt), especially the N-terminal-cleaved Htt, participates in the pathophysiology of Huntington's disease (HD). it is difficult to elucidate temporal properties of the translocation of endogenous Htt using autopsy HD patient brains. Thus, we examined the cell biology of endogenous Htt cleavage and nuclear translocation in cultured lymphoblasts of HD patients and controls. Apoptotic stimulation of lymphoblasts elicits caspase-dependent cleavage and selective nuclear translocation of N-terminal portions of Htt. Discrete clusters of the N-terminal Hit accumulate at unique perinuclear sites prior to nuclear translocation. Our findings suggest that caspase cleavage of Htt is cytoplasmic and precedes sorting to specific perinuclear sites followed by nuclear translocation in HD patient tissue. (c) 2005 Elsevier Inc. All rights reserved.
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