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Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death

Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death
Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death
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.
huntington's disease, oculopharyngeal muscular dystrophy, polyglutamine, trinucleotide
1470-8728
15-19
Rankin, Julia
e10a0f80-2929-4b47-8fa2-8dee01ce39c6
Wyttenbach, Andreas
05019897-52b1-4bb6-b259-5d51abae7540
Rubinsztein, David C.
c841997b-4a3f-4643-b215-0f206d29a39e
Rankin, Julia
e10a0f80-2929-4b47-8fa2-8dee01ce39c6
Wyttenbach, Andreas
05019897-52b1-4bb6-b259-5d51abae7540
Rubinsztein, David C.
c841997b-4a3f-4643-b215-0f206d29a39e

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.

Record type: Article

Abstract

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.

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More information

Published date: 2000
Keywords: huntington's disease, oculopharyngeal muscular dystrophy, polyglutamine, trinucleotide

Identifiers

Local EPrints ID: 55992
URI: https://eprints.soton.ac.uk/id/eprint/55992
ISSN: 1470-8728
PURE UUID: 50aadc83-4290-4a7f-bab3-80f716e5014b

Catalogue record

Date deposited: 20 Aug 2008
Last modified: 17 Jul 2017 14:31

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