IndraLab

Statements


Mutated ATXN3 activates apoptotic process. 4 / 4
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"Inactivation of PNKP by Mutant ATXN3 Triggers Apoptosis by Activating the DNA Damage Response Pathway in SCA3."

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"Injection of mutant ATXN3 mRNA containing a CAG repeat expansion encoding ATXN3[80Q] caused p53-dependent apoptosis that was mainly detected in the head of the zebrafish at 24hpf, possibly indicating neuronal degeneration [71]."

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"In cell and animal models of SCA3 induced by ataxin-3 mutation, mutant SCA3 can induce apoptosis of cerebellum and pons neurons by enhancing p53 transcriptional activity (Chou et al., 2011)."

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"Since it is well known that Bcl-2 inhibits cell death induced by a variety of stimuli, mutant ataxin-3 may cause neuronal apoptosis through a down-regulation of Bcl-2, but not an up-regulation of Bax."