Polyglutamine-expanded ataxin-1 recruits Cu/Zn-superoxide dismutase into the nucleus of HeLa cells

Sung Jo Kim, Tae Soo Kim, Ick Young Kim, Sunghoi Hong, Hyangshuk Rhim, Seong Man Kang

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Spinocerebellar ataxia 1 (SCA1) is an inherited neurodegenerative disorder caused by expansion of the polyglutamine stretch in ataxin-1, the SCA1 gene product. Polyglutamine expansion leads to the aggregation of ataxin-1 proteins. Superoxide dismutases (SODs) are involved in the pathogenesis of other aggregate-forming neurodegenerative diseases and are known to localize in the cytoplasm. Here, we show that Cu/Zn-SOD is translocated into the nucleus of HeLa cells in the presence of expanded ataxin-1, whereas Mn-SOD is localized in the cytoplasm: the longer the expansion of polyglutamine, the higher the level of translocation of Cu/Zn-SOD. In addition, the oxidation of intracellular proteins occurs with higher frequency in the presence of mutant ataxin-1 (82Q), suggesting that the functional activity of Cu/Zn-SOD might be decreased by mutant ataxin-1. We demonstrate that mutant ataxin-1-expressing cells encounter mitochondrial dysfunction in the conditions of oxidative stress. Our results suggest that polyglutamine-expanded ataxin-1 increases the levels of reactive oxygen species in HeLa cells.

Original languageEnglish
Pages (from-to)660-665
Number of pages6
JournalBiochemical and Biophysical Research Communications
Volume307
Issue number3
DOIs
Publication statusPublished - 2003 Aug 1

Fingerprint

HeLa Cells
Spinocerebellar Ataxias
Neurodegenerative Diseases
Superoxide Dismutase
Cytoplasm
Neurodegenerative diseases
Oxidative stress
Superoxide Dismutase-1
Ataxin-1
polyglutamine
Reactive Oxygen Species
Oxidative Stress
Agglomeration
Genes
Oxidation
Proteins

Keywords

  • Aggregate
  • Polyglutamine
  • Reactive oxygen species
  • SCA1
  • Superoxide dismutase

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

Cite this

Polyglutamine-expanded ataxin-1 recruits Cu/Zn-superoxide dismutase into the nucleus of HeLa cells. / Kim, Sung Jo; Kim, Tae Soo; Kim, Ick Young; Hong, Sunghoi; Rhim, Hyangshuk; Kang, Seong Man.

In: Biochemical and Biophysical Research Communications, Vol. 307, No. 3, 01.08.2003, p. 660-665.

Research output: Contribution to journalArticle

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