p80 coilin, a coiled body-specific protein, interacts with ataxin-1, the SCA1 gene product

Sunghoi Hong, Sojeong Ka, Sungjo Kim, Yongjae Park, Seong Man Kang

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder characterized by ataxia and progressive motor deterioration. SCA1 is associated with an elongated polyglutamine tract in ataxin-1, the SCA1 gene product. Using the yeast two-hybrid system and co-immunoprecipitation experiments, we have found that p80 coilin, coiled body-specific protein, binds to ataxin-1. In further experiments with deletion mutants, we found that the C-terminal regions of ataxin-1 and p80 coilin were essential for this interaction. In HeLa cells that have been co-transfected with ataxin-1 and p80 coilin, the p80 coilin protein co-localizes with ataxin-1 aggregates in the nucleoplasm. However, immunohistochemical analysis and immunofluorescence assays showed that mutant ataxin-1 aggregates do not redistribute p80 coilin's dot-like structures in the Purkinje cells of SCA1 transgenic mice. This feature of the interaction between ataxin-1 and p80 coilin suggests that p80 coilin might be implicated in altering the function of ataxin-1.

Original languageEnglish
Pages (from-to)35-42
Number of pages8
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1638
Issue number1
DOIs
Publication statusPublished - 2003 May 20

Fingerprint

Coiled Bodies
Spinocerebellar Ataxias
Genes
Proteins
p80-coilin
Ataxin-1
Two-Hybrid System Techniques
Purkinje Cells
Ataxia
HeLa Cells
Immunoprecipitation
Neurodegenerative Diseases
Transgenic Mice
Fluorescent Antibody Technique

Keywords

  • Coiled body
  • p80 coilin
  • Polyglutamine
  • SCA1
  • Yeast two-hybrid

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine
  • Biophysics

Cite this

p80 coilin, a coiled body-specific protein, interacts with ataxin-1, the SCA1 gene product. / Hong, Sunghoi; Ka, Sojeong; Kim, Sungjo; Park, Yongjae; Kang, Seong Man.

In: Biochimica et Biophysica Acta - Molecular Basis of Disease, Vol. 1638, No. 1, 20.05.2003, p. 35-42.

Research output: Contribution to journalArticle

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