IndraLab

Statements

ATXN2 activates ATXN3. 14 / 14
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"SCA2 and SCA3 are caused by polyglutamine expansions in ataxin2 and ataxin3, respectively [XREF_BIBR, XREF_BIBR]."
20383523
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"Up-regulation of atx2 synergistically enhanced SCA3 degeneration, and strikingly, we found that the endogenous activity of atx2 modulates progression of neurodegeneration induced by pathogenic Atx3."
18271626
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"There are also 6 types of spinocerebellar ataxias (SCA1, SCA2, SCA3, SCA6, SCA7, SCA17), which are caused by expansions in the genes ATXN1 , ATXN2 , ATXN3 , CACNA1A , ATXN7 and TBP , respectively."
30597161
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"These observations indicated that PABP has the opposite activity as Atx2 with respect to Atx3 dependent neurodegeneration : whereas Atx2 enhances the toxicity of Atx3, PABP is protective."
18271626
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"In the fly, endogenous Atx2 colocalized with pathogenic Atx3 in inclusions, as seen in human patients [XREF_BIBR], with up-regulation of Atx2 enhancing Atx3 toxicity concomitant with a faster onset of inclusions and of SDS-insoluble complexes."
18271626
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"XREF_BIBR SCA1, SCA2, Machado-Joseph or SCA3, SCA6, SCA7, SCA12, SCA17, and dentatorubral-pallidoluysian atrophy (DRPLA) are caused by (CAG) n repeat expansions in the ATXN1, ATXN2, ATXN3, CACNA1A, ATXN7, PPP2R2B, TBP, and ATN1 genes, respectively, and all lead to the expansion of a polyglutamine tract in the corresponding proteins."
24780882
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"Up-Regulation of Atx2 Synergistically Enhances Atx3 Induced Neurodegeneration."
18271626
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"At the 24-h time point, coexpression of normal Atx2 with pathogenic Atx3 caused the early appearance of SDS-insoluble Atx3 complexes that remain within the stacking gel (compare lanes 1 and 2), presumably reflecting protein accumulations that correlate with the early appearance of nuclear inclusions in cryosections."
18271626
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"Ataxin gene variants in ATXN1, ATXN2, ATXN3, and ATXN7 cause SCA1, SCA2, SCA3, and SCA7, respectively."
29168350
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"Atx-2 was found to mediate the pathogenic effects of SCA-1 and SCA-3."
19373559
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"Intermediate-length ATXN2 polyQ (29-to-31Q) modulate AO in SCA3/MJD and is a proven disease modifier for C9ORF72-ALS (van Blitterswijk et al., 2014; de Mattos et al., 2019)."
35535893
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"SCA1, SCA2, and SCA3 are caused by large abnormal CAG trinucleotide repeat expansions in ATXN1, ATXN2, and ATXN3 genes, respectively (Chung et al., 1993; Orr et al., 1993; Kawaguchi et al., 1994; Pulst et al., 1996)."
37397792
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"Moreover, up-regulation of atx2, the Drosophila ortholog of the human gene that causes SCA2 disease, has been shown to enhance the toxicity of human disease forms of SCA1 and SCA3 in flies [XREF_BIBR]."
18271626
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"Our findings demonstrated that up-regulation of Atx2 activity modulates SCA3 pathogenesis."
18271626