title: Ataxin-2-like is a regulator of stress granules and processing bodies.
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.
authors.affiliation: Otto Warburg Laboratory, Max Planck Institute for Molecular Genetics, Berlin, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Kaehler
authors.forename: Jörg
authors.initials: J
authors.lastname: Isensee
authors.forename: Ute
authors.initials: U
authors.lastname: Nonhoff
authors.forename: Markus
authors.initials: M
authors.lastname: Terrey
authors.forename: Tim
authors.initials: T
authors.lastname: Hucho
authors.forename: Hans
authors.initials: H
authors.lastname: Lehrach
authors.forename: Sylvia
authors.initials: S
authors.lastname: Krobitsch
mesh_terms: D000067528:Ataxins; D045744:Cell Line, Tumor; D003593:Cytoplasm / Q000378:metabolism; D053487:DEAD-box RNA Helicases / Q000378:metabolism; D005786:Gene Expression Regulation; D057809:HEK293 Cells; D006367:HeLa Cells; D006801:Humans; D018613:Microscopy, Confocal / Q000379:methods; D008856:Microscopy, Fluorescence / Q000379:methods; D008954:Models, Biological; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D039122:Poly(A)-Binding Protein I / Q000378:metabolism; D039102:Poly(A)-Binding Proteins / Q000378:metabolism*; D011518:Proto-Oncogene Proteins / Q000378:metabolism; D012313:RNA / Q000378:metabolism; D034622:RNA Interference; D015398:Signal Transduction; D020754:Spinocerebellar Ataxias / Q000378:metabolism
chemical_list: D000067528:Ataxins; D009419:Nerve Tissue Proteins; D039122:Poly(A)-Binding Protein I; D039102:Poly(A)-Binding Proteins; D011518:Proto-Oncogene Proteins; D012313:RNA; C094683:DDX6 protein, human; D053487:DEAD-box RNA Helicases