IndraLab

Statements

Mutated ATXN3 activates Death. 4 / 4
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"Since the mechanism by which mutant ataxin-3 eventually leads to neuronal death is poorly understood, additional investigations to clarify the biological alterations related to Machado-Joseph disease are necessary."
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"Moreover, amelioration of ATXN3-Q84-mediated phosphorylation of p53, c-Abl and PKCdelta by pharmacological inhibition of ATM suggests that mutant ATXN3 activates the pro death signaling cascades via activating ATM in SCA3."
25590633
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"These data substantiate our previous interpretation that mutant ATXN3 activates the p53 dependent pro death pathway by activating ATM, and that chronic activation of the ATM --> p53 pathway plays a pivotal role in mediating neuronal death in SCA3."
25590633
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"The susceptibility to death induced by the expression of mutant ataxin-3 varies among individual cell types, even among neurons [14]."
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