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Expression and Characterization of Full-length Human Huntingtin, an Elongated HEAT Repeat Protein.
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posted on 2023-06-07, 21:35 authored by Wei Li, Louise SerpellLouise Serpell, Wendy J Carter, David C Rubinsztein, James A HuntingtonHuntington 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.
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Publication status
- Published
Journal
Journal of Biological ChemistryISSN
0021-9258External DOI
Issue
23Volume
281Page range
15916-15922Department affiliated with
- Biochemistry Publications
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- No
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- Yes
Legacy Posted Date
2012-02-06Usage metrics
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