Ataxin-2-like is a regulator of stress granules and processing bodies

PLoS One. 2012;7(11):e50134. doi: 10.1371/journal.pone.0050134. Epub 2012 Nov 27.

Abstract

Paralogs for several proteins implicated in neurodegenerative disorders have been identified and explored to further facilitate the identification of molecular mechanisms contributing to disease pathogenesis. For the disease-causing protein in spinocerebellar ataxia type 2, ataxin-2, a paralog of unknown function, termed ataxin-2-like, has been described. We discovered that ataxin-2-like associates with known interaction partners of ataxin-2, the RNA helicase DDX6 and the poly(A)-binding protein, and with ataxin-2 itself. Furthermore, we found that ataxin-2-like is a component of stress granules. Interestingly, sole ataxin-2-like overexpression led to the induction of stress granules, while a reduction of stress granules was detected in case of a low ataxin-2-like level. Finally, we observed that overexpression of ataxin-2-like as well as its reduction has an impact on the presence of microscopically visible processing bodies. Thus, our results imply a functional overlap between ataxin-2-like and ataxin-2, and further indicate a role for ataxin-2-like in the regulation of stress granules and processing bodies.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ataxins
  • Cell Line, Tumor
  • Cytoplasm / metabolism
  • DEAD-box RNA Helicases / metabolism
  • Gene Expression Regulation*
  • HEK293 Cells
  • HeLa Cells
  • Humans
  • Microscopy, Confocal / methods
  • Microscopy, Fluorescence / methods
  • Models, Biological
  • Nerve Tissue Proteins / metabolism*
  • Poly(A)-Binding Protein I / metabolism
  • Poly(A)-Binding Proteins / metabolism*
  • Proto-Oncogene Proteins / metabolism
  • RNA / metabolism
  • RNA Interference
  • Signal Transduction
  • Spinocerebellar Ataxias / metabolism

Substances

  • Ataxins
  • Nerve Tissue Proteins
  • Poly(A)-Binding Protein I
  • Poly(A)-Binding Proteins
  • Proto-Oncogene Proteins
  • RNA
  • DDX6 protein, human
  • DEAD-box RNA Helicases

Grants and funding

This work was supported by the Max Planck Society. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.