Gene Symbol: Atx2
Description: Ataxin-2
Alias: ATX2, Atx-2, Atxain-2, BEST:GH27029, CG5166, CG5166a, Datx2, Dmel\CG5166, GH27029, SCA2, Sca2, anon-EST:fe2E1, anon-WO0118547.351, anon-WO0140519.1, atx-2, atx2, dAtx2, dSCA2, l(3)06490, Ataxin-2, ATAXIN-2, Atx2-PA, Atx2-PB, Atx2-PC, Atx2-PD, CG5166-PA, CG5166-PB, CG5166-PC, CG5166-PD, anon-fast-evolving-2E1, ataxin 2, dAtaxin-2
Species: fruit fly

Top Publications

  1. Elden A, Kim H, Hart M, Chen Plotkin A, Johnson B, Fang X, et al. Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature. 2010;466:1069-75 pubmed publisher
    ..Here we show that ataxin 2 (ATXN2), a polyglutamine (polyQ) protein mutated in spinocerebellar ataxia type 2, is a potent modifier of TDP-43 ..
  2. Latouche M, Lasbleiz C, Martin E, Monnier V, Debeir T, Mouatt Prigent A, et al. A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes. J Neurosci. 2007;27:2483-92 pubmed
    ..Sodium butyrate, a histone deacetylase inhibitor, improved the survival time of the neurons. This model is therefore a powerful tool for studying SCA7 and for the development of potential therapies for polyQ diseases. ..
  3. Satterfield T, Jackson S, Pallanck L. A Drosophila homolog of the polyglutamine disease gene SCA2 is a dosage-sensitive regulator of actin filament formation. Genetics. 2002;162:1687-702 pubmed
    ..have used genetic and molecular approaches to investigate the function of a Drosophila homolog of the SCA2 gene (Datx2)...
  4. Lessing D, Bonini N. Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila. PLoS Biol. 2008;6:e29 pubmed publisher
    ..due to SCA3 in Drosophila, we isolated atx2, the fly ortholog of the human gene that causes a related ataxia, SCA2. We show that the normal activity of Ataxin-2 (Atx2) is critical for SCA3 degeneration and that Atx2 activity ..
  5. Al Ramahi I, Perez A, Lim J, Zhang M, Sorensen R, de Haro M, et al. dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet. 2007;3:e234 pubmed publisher
    ..We show that wild-type Drosophila Ataxin-2 (dAtx2) is a major genetic modifier of human expanded Ataxin-1 (Ataxin-1[82Q]) toxicity...
  6. Satterfield T, Pallanck L. Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes. Hum Mol Genet. 2006;15:2523-32 pubmed
    ..Here, we demonstrate that ataxin-2 and its Drosophila homolog, ATX2, assemble with polyribosomes and poly(A)-binding protein (PABP), a key regulator of mRNA translation...
  7. Ghosh S, Feany M. Comparison of pathways controlling toxicity in the eye and brain in Drosophila models of human neurodegenerative diseases. Hum Mol Genet. 2004;13:2011-8 pubmed
    ..These findings outline common pathways of neurotoxicity, demonstrate disease- and cell-type specific pathways and identify a common vitamin as a potential therapy in polyglutamine disorders. ..
  8. Bonini N, Gitler A. Model organisms reveal insight into human neurodegenerative disease: ataxin-2 intermediate-length polyglutamine expansions are a risk factor for ALS. J Mol Neurosci. 2011;45:676-83 pubmed publisher
    ..that repeat lengths are greater than normal, but lower than that associated with spinocerebellar ataxia type 2 (SCA2), and are more frequent in ALS patients than in matched controls...
  9. Lawlor K, O Keefe L, Samaraweera S, van Eyk C, McLeod C, Maloney C, et al. Double-stranded RNA is pathogenic in Drosophila models of expanded repeat neurodegenerative diseases. Hum Mol Genet. 2011;20:3757-68 pubmed publisher
    ..These data identify a novel pathway through which double-stranded repeat RNA is toxic and capable of eliciting symptoms common to neurodegenerative human diseases resulting from dominantly inherited expanded repeats. ..

More Information


  1. Costa A, Pazman C, Sinsimer K, Wong L, McLeod I, Yates J, et al. Rasputin functions as a positive regulator of orb in Drosophila oogenesis. PLoS ONE. 2013;8:e72864 pubmed publisher
    ..Tandem Mass Spectrometry analysis shows that several canonical stress granule proteins are associated with the Orb-Rin complex suggesting that a conserved mRNP complex regulates localized translation during oogenesis in Drosophila. ..
  2. Ruby J, Jan C, Bartel D. Intronic microRNA precursors that bypass Drosha processing. Nature. 2007;448:83-6 pubmed
    ..This suggests that other lineages with many similarly sized introns probably also have mirtrons, and that the mirtron pathway could have provided an early avenue for the emergence of miRNAs before the advent of Drosha. ..
  3. Sun X, Morozova T, Sonnenfeld M. Glial and neuronal functions of the Drosophila homolog of the human SWI/SNF gene ATR-X (DATR-X) and the jing zinc-finger gene specify the lateral positioning of longitudinal glia and axons. Genetics. 2006;173:1397-415 pubmed
  4. Tastan O, Maines J, Li Y, McKearin D, Buszczak M. Drosophila ataxin 2-binding protein 1 marks an intermediate step in the molecular differentiation of female germline cysts. Development. 2010;137:3167-76 pubmed publisher
    ..Here, we report that the early development of germline cysts depends on the Drosophila homolog of the human ataxin 2-binding protein 1 (A2BP1) gene...
  5. Kumar S. Cell death in the fly comes of age. Cell Death Differ. 2000;7:1021-4 pubmed
  6. McCann C, Holohan E, Das S, Dervan A, Larkin A, Lee J, et al. The Ataxin-2 protein is required for microRNA function and synapse-specific long-term olfactory habituation. Proc Natl Acad Sci U S A. 2011;108:E655-62 pubmed publisher
    ..local interneurons observed during long-term olfactory habituation (LTH) requires the Ataxin-2 protein (Atx2) to function in uniglomerular projection neurons (PNs) postsynaptic to local interneurons (LNs)...
  7. Sudhakaran I, Hillebrand J, Dervan A, Das S, Holohan E, H lsmeier J, et al. FMRP and Ataxin-2 function together in long-term olfactory habituation and neuronal translational control. Proc Natl Acad Sci U S A. 2014;111:E99-E108 pubmed publisher
    Fragile X mental retardation protein (FMRP) and Ataxin-2 (Atx2) are triplet expansion disease- and stress granule-associated proteins implicated in neuronal translational control and microRNA function...
  8. Toret C, D Ambrosio M, Vale R, Simon M, Nelson W. A genome-wide screen identifies conserved protein hubs required for cadherin-mediated cell-cell adhesion. J Cell Biol. 2014;204:265-79 pubmed publisher
    ..These experiments reveal roles for a diversity of cellular pathways that are required for cadherin function in Metazoa, including cytoskeleton organization, cell-substrate interactions, and nuclear and cytoplasmic signaling. ..
  9. Lim C, Allada R. ATAXIN-2 activates PERIOD translation to sustain circadian rhythms in Drosophila. Science. 2013;340:875-9 pubmed publisher
    ..We found that ATAXIN-2 (ATX2), an RNA-associated protein involved in neurodegenerative disease, is a translational activator of the rate-..
  10. Zhang Y, Ling J, Yuan C, Dubruille R, Emery P. A role for Drosophila ATX2 in activation of PER translation and circadian behavior. Science. 2013;340:879-82 pubmed publisher
    ..We found that the Drosophila homolog of ATAXIN-2 (ATX2)--an RNA-binding protein implicated in human neurodegenerative diseases--was required for circadian locomotor ..
  11. Na D, Rouf M, O Kane C, Rubinsztein D, Gsponer J. NeuroGeM, a knowledgebase of genetic modifiers in neurodegenerative diseases. BMC Med Genomics. 2013;6:52 pubmed publisher
    ..For instance, we reveal that the Drosophila genes DnaJ-1, thread, Atx2, and mub are generic modifiers that affect multiple if not all NDs...
  12. Lee J, Yoo E, Lee H, Park K, Hur J, Lim C. LSM12 and ME31B/DDX6 Define Distinct Modes of Posttranscriptional Regulation by ATAXIN-2 Protein Complex in Drosophila Circadian Pacemaker Neurons. Mol Cell. 2017;66:129-140.e7 pubmed publisher
    ATAXIN-2 (ATX2) has been implicated in human neurodegenerative diseases, yet it remains elusive how ATX2 assembles specific protein complexes to execute its physiological roles...
  13. Yu Z, Teng X, Bonini N. Triplet repeat-derived siRNAs enhance RNA-mediated toxicity in a Drosophila model for myotonic dystrophy. PLoS Genet. 2011;7:e1001340 pubmed publisher
    ..These findings indicate that the generation of triplet repeat-derived siRNAs may dramatically enhance toxicity in human repeat expansion diseases in which anti-sense transcription occurs...