IndraLab

Statements


ATXN2 activates ATXN3. 11 / 11
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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."

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"Atx-2 was found to mediate the pathogenic effects of SCA-1 and SCA-3."

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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."

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"Ataxin gene variants in ATXN1, ATXN2, ATXN3, and ATXN7 cause SCA1, SCA2, SCA3, and SCA7, respectively."

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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."

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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."

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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."

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"Up-Regulation of Atx2 Synergistically Enhances Atx3 Induced Neurodegeneration."

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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]."

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"SCA2 and SCA3 are caused by polyglutamine expansions in ataxin2 and ataxin3, respectively [XREF_BIBR, XREF_BIBR]."

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"Our findings demonstrated that up-regulation of Atx2 activity modulates SCA3 pathogenesis."