IndraLab

Statements

Mutated ATXN3 activates TP53. 6 / 6
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"Mutant ATXN3 activates the pro apoptotic p53 pathway by activating ATM in SCA3."
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"Specific modulation of mutant ATXN3 mediated atypical activation of the DNA damage response p53 and PKCdelta pathways, or enhancing the efficacy of in vivo DNA damage repair may be effective strategies to combat the pathways leading to systemic neurodegeneration in SCA3."
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"Knockdown of normal ATXN3 or PNKP, or expression of mutant ATXN3 increased the accumulation of DNA damage and persistent activation of the ATM and p53 dependent DNA repair pathway, suggesting ATXN3 may be a key regulator of PNKP dependent DNA repair."
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"Notably, compared with normal ataxin-3 protein, the gain-of-function mutant ataxin-3 triggers higher p53 protein and p53 responsive gene expression and causes more severe p53 dependent neurodegeneration in brain neuron cells of transgenic zebrafish and SCA3 mice 13."
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"Furthermore, to test whether mutant ATXN3 activates p53 and Chk2 via activating ATM, we pre-treated the cells with ATM inhibitor Ku55933 and expressed ATXN3-Q84 and assessed the activation of DNA damage response pathway."
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"Consistent with our hypothesis, ATXN3-Q84 expression failed to stimulate phosphorylation of Chk2 and p53 in the presence of the ATM inhibitor Ku55933 (XREF_SUPPLEMENTARY), substantiating our interpretation that mutant ATXN3 stimulates the DNA damage response p53 pathway via activating ATM."
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