Recombinant Mouse Ataxin-3 (Atxn3)

1. findings identify a novel molecular link between ATX-3 and p53-mediated cell death and provide an explanation for the direct involvement of p53 in SCA3 disease pathogenesis PMID: 27851749
2. We show that chronic VPA treatment did not modify the ATXN3 inclusion load and astrogliosis in affected brain regions However, VPA chronic treatment was able to increase GRP78 protein levels at 30 weeks of age, one of its known neuroprotective effects PMID: 26505994
3. work suggests that in Machado-Joseph disease, mutant ataxin-3 drives an abnormal reduction of ataxin-2 levels, which overactivates poly(A)-binding protein, increases translation of mutant ataxin-3 and other proteins and aggravates Machado-Joseph disease. PMID: 26490332
4. SCA3 knockin mice exhibit robust Atxn3 accumulation both in regions known to be affected in human disease; also display altered splicing of the mutant Atxn3 transcript that results in the formation of a previously described alternative ATXN3 transcript PMID: 25320121
5. Data support the importance of ATXN3 in neuronal cells and indicate that an expanded polyQ tract leads to a partial loss of the cellular function of ATXN3 that may be relevant to neurodegeneration. PMID: 25143392
6. While ataxin-3 may participate in protein quality control pathways, it does not critically regulate the handling of mutant htt or contribute to major features of disease pathogenesis in Huntington disease. PMID: 24683430
7. Results suggest that postnatal nuclear accumulation of mutant ataxin-3 disrupts dendritic differentiation and mGluR-signaling in mouse model spinocerebellar ataxia type 3 Purkinje cells PMID: 23955261
8. Lentiviral-based expression of mutant atxn-3 in the mouse cerebellum induces localized neuropathology sufficient to generate a behavioral ataxic phenotype. PMID: 23242710
9. Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice. PMID: 23626768
10. the efficacy of gene silencing in blocking the MJD-associated motor-behavior and neuropathological abnormalities PMID: 23349684
11. the sequestration of misfolded SOD1 into aggresomes, which is driven by ataxin-3, plays an important role in attenuating protein misfolding-induced cell toxicity. PMID: 22761419
12. Human calpastatin promotes neuroprotection by decreasing mutant ataxin 3 fragment production in transgenic mice. PMID: 22843411
13. we propose that -1 ribosomal frameshifting contributes to the toxicity associated with (exp)CAG repeats. PMID: 22337953
14. an unexpected convergence upon the E2 Ub-conjugating enzyme in the regulation of an E3/deubiquitinating enzyme pair, with important implications for the function of parkin and ataxin-3 PMID: 22081612
15. This study demonistrated that n-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation in mice. PMID: 21653538
16. Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117 PMID: 20943656
17. Data show that the absence of ATXN3 leads to an overt cytoskeletal/adhesion defect raising the possibility that this protein may play a role in the cytoskeleton. PMID: 20637808
18. Ataxin-3 is important for myogenesis through regulation of integrin subunit levels. PMID: 20668528
19. CK2 and GSK3 phosphorylation on S29 controls wild-type ATXN3 nuclear uptake. PMID: 20347968
20. We therefore conclude that overexpressing wild type ataxin-3 or mutant ataxin-3 with NES are not striking suppressors of polyglutamine-induced neurodegeneration and have thus no potential for future gene therapeutic interventions in SCA3. PMID: 20079840
21. native aggregation-prone fragments derived from expanded ataxin-3 may eventually escape the cytoplasmic quality control, resulting in aggregation in the nuclear compartment. PMID: 20064935
22. Oxidative stress and heat shock induced nuclear localization of Atx3. PMID: 19843543
23. Gp78 promotes SOD1 and ataxin-3 degradation in endoplasmic reticulum. PMID: 19661182
24. ataxin-3 human and mouse protein sequences share a highly conserved N-terminus and differ only in the length of the glutamine repeats and in the C-terminus; the domain architecture of both is detailed PMID: 12914917
25. At temperatures to 96 degrees C, unlike the human polyglutaminated form, mouse ataxin-3 does not display large structural changes, nor does it undergo any precipitation, which highlights its amazing heat-resistance. PMID: 14661975
26. The mouse Mjd gene has a structure similar to that of its human counterpart; it is ubiquitously expressed during embryonic development and in the adult, with strong expression in regions of the CNS affected in MJD. PMID: 15233999
27. This article shows that Ataxin-3 also has autoproteolytic activity, sustained by the same residues responsible for the ubiquitin hydrolase activity. PMID: 16939621
28. ataxin-3 proteolysis in neuroblastoma cells and in vitro and show that calcium-dependent calpain proteases generate aggregation-competent ataxin-3 fragments PMID: 17488727
29. We identified one positive case of SCA3 among those who were diagnosed with ET, yielding a prevalence of 0.5%, but a zero prevalence among our atypical Parkinsonism patients. PMID: 17712857
30. Atxn3 (ataxin-3) gene has a role in regulating protein ubiquitination PMID: 17764659
31. polyglutamine-expanded ataxin-3 causes cerebellar dysfunction and ataxia by disrupting the normal pattern of gene transcriptions PMID: 18502140
32. the formation of AT3 aggregation may affect the normal function of WT AT3 and increase polyQ protein toxicity in Machado-Joseph disease PMID: 18668148

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KEGG: mmu:110616

STRING: 10090.ENSMUSP00000021606

UniGene: Mm.271914