Journal
HUMAN MOLECULAR GENETICS
Volume 14, Issue 6, Pages 765-774Publisher
OXFORD UNIV PRESS
DOI: 10.1093/hmg/ddi071
Keywords
CMV infection; CMV disease; CMV prophylaxis; CMV monitoring; kidney transplantation
Funding
- NINDS NIH HHS [NS16375] Funding Source: Medline
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Huntington's disease (HD) arises from an expanded polyglutamine (polyQ) in the N-terminus of the huntingtin (htt) protein. Neuronal degeneration and inclusions containing N-terminal fragments of mutant htt are present in the cortex and striatum of HD brain. Recently, a model of polyQ aggregate structure has been proposed on the basis of studies with synthetic polyQ peptides and includes an alternating beta-strand/ beta-turn structure with seven glutamine residues per beta-strand. We tested this model in the context of the htt exon- 1 N- terminal fragment in both mammalian cell culture and cultured primary cortical neurons. We found our data support this model in the htt protein and provide a better understanding of the structural basis of polyQ aggregation in toxicity in HD.
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