IndraLab

Statements


Mutated ATXN3 activates cell death. 6 / 6
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"A neuroendocrine dysfunction, not testicular mutant ataxin-3 cleavage fragment or aggregate, causes cell death in testes of transgenic mice."

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"The mutant ataxin-3 forms intranuclear inclusions in cultured cells as well as in diseased human brain and also causes cell death in transfected cells."

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"Moreover, overexpression of PNKP in these cells blocked ATXN3-Q84-mediated caspase-3 activation (XREF_FIG), suggesting that the loss of PNKP function plays an important role in mutant ATXN3 mediated cell death."

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"Either PNKP overexpression or pharmacological inhibition of ATM dramatically blocked mutant ATXN3 mediated cell death."

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"In SCA3, full length mutant ATXN3 has been shown to increase mitochondrial mediated cell death in different models."

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"Coexpression of p21 (waf1/cip1/sdi1), a cyclin-Cdk inhibitor that induced cell cycle arrest in the G (1) phase, also increased the cell death susceptibility produced by the mutant ataxin-3 fragment in BHK-21 cells."