Expression and Characterization of Full-length Human Huntingtin, an Elongated HEAT Repeat Protein.

Li, Wei, Serpell, Louise C, Carter, Wendy J, Rubinsztein, David C and Huntington, James A (2006) Expression and Characterization of Full-length Human Huntingtin, an Elongated HEAT Repeat Protein. Journal of Biological Chemistry, 281 (23). pp. 15916-15922. ISSN 0021-9258

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Abstract

Huntington disease is an inherited neurodegenerative disorder that is caused by expanded CAG trinucleotide repeats, resulting in a polyglutamine stretch of >37 on the N terminus of the protein huntingtin (htt). htt is a large (347 kDa), ubiquitously expressed protein. The precise functions of htt are not clear, but its importance is underscored by the embryonic lethal phenotype in htt knock-out mice. Despite the fact that the htt gene was cloned 13 years ago, little is known about the properties of the full-length protein. Here we report the expression and preliminary characterization of recombinant full-length wild-type human htt. Our results support a model of htt composed entirely of HEAT repeats that stack to form an elongated superhelix.

Item Type: Article
Schools and Departments: School of Life Sciences > Biochemistry
Depositing User: Louise Serpell
Date Deposited: 06 Feb 2012 18:51
Last Modified: 20 Mar 2012 12:18
URI: http://sro.sussex.ac.uk/id/eprint/18675
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