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- Title
SUMO-1 Modification on K166 of PolyQ-Expanded aTaxin-3 Strengthens Its Stability and Increases Its Cytotoxicity.
- Authors
Ya-Fang Zhou; Shu-Sheng Liao; Ying-Ying Luo; Jian-Guang Tang; Jun-Ling Wang; Li-Fang Lei; Jing-Wei Chi; Juan Du; Hong Jiang; Kun Xia; Bei-Sha Tang; Lu Shen
- Abstract
Post-translational modification by SUMO was proposed to modulate the pathogenesis of several neurodegenerative diseases. Spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD) is an autosomal dominant neurodegenerative disease caused by polyQ-expanded ataxin-3. We have previously shown that ataxin-3 was a new target of SUMOylation in vitro and in vivo. Here we identified that the major SUMO-1 binding site was located on lysine 166. SUMOylation did not influence the subcellular localization, ubiquitination or aggregates formation of mutant-type ataxin-3, but partially increased its stability and the cell apoptosis. Our findings revealed the role of ataxin-3 SUMOylation in SCA3/MJD pathogenesis.
- Publication
PLoS ONE, 2013, Vol 8, Issue 1, p1
- ISSN
1932-6203
- Publication type
Academic Journal
- DOI
10.1371/journal.pone.0054214