Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death
Rankin, Julia, Wyttenbach, Andreas and Rubinsztein, David C. (2000) Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death. Biochemical Journal, 348, 15-19.
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Eight diseases, exemplified by Huntington's disease and spinocerebellar ataxia type 1, are caused by CAG-repeat expansion mutations. The CAG repeats are translated into expanded polyglutamine tracts, which are associated with deleterious novel functions. While these diseases are characterized by intraneuronal aggregate formation, it is unclear whether the aggregates cause disease. We have addressed this debate by generating intracellular aggregates with green fluorescent protein (GFP) fused to 19–37 alanines. No aggregates were seen in cells expressing native GFP or GFP fused to seven alanines. Aggregate-containing cells expressing GFP fused to 19–37 polyalanines show high rates of nuclear fragmentation compared with cells expressing the same constructs without aggregates, or cells expressing GFP fused to seven alanines. This suggests an association between aggregate formation and cell death.
|Keywords:||huntington's disease, oculopharyngeal muscular dystrophy, polyglutamine, trinucleotide|
|Subjects:||R Medicine > R Medicine (General)
Q Science > QH Natural history > QH301 Biology
|Divisions:||University Structure - Pre August 2011 > School of Biological Sciences
|Date Deposited:||20 Aug 2008|
|Last Modified:||01 Jun 2011 09:37|
|Contributors:||Rankin, Julia (Author)
Wyttenbach, Andreas (Author)
Rubinsztein, David C. (Author)
|RDF:||RDF+N-Triples, RDF+N3, RDF+XML, Browse.|
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