IndraLab

Statements

Mutated ATXN3 inhibits PNKP. 7 / 7
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"Conversely, polyQ expanded mutant ATXN3 inhibited PNKP activity, possibly as a result of PNKP sequestration into ATXN3 aggregates."
31669734
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"In conclusion, our current study provides compelling evidence of how mutant ATXN3 impedes the enzymatic activity of PNKP, and induces DNA damage that manifests with activation of the pro apoptotic signaling pathways in SCA3."
25590633
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"It has been suggested that the PNKP inactivation by mutant ataxin-3 might in part be explained by its recruitment in polyQ aggregates XREF_BIBR."
27731380
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"Mutant ATXN3 has also been found to sequester PNKP outside the nucleus and thereby impair its ability to take part in DNA repair [XREF_BIBR]."
33669593
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"The wild-type ATXN3 protein stimulated, and in contrast, the mutant ATXN3 dramatically diminished, the 3 ' phosphatase activity of PNKP in vitro (Chatterjee et al; Figs."
25590633
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"Here we report that WT ATXN3 stimulates, and by contrast mutant ATXN3 blocks the DNA 3 '-end-processing activity of PNKP, and the resulting accumulation of DNA SBs may contribute significantly to SCA3 pathogenesis via modulating the DNA damage response pathway."
25633985
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"These data further support the idea that the mutant ATXN3 specifically blocks the activity of PNKP, but not that of DNA polymerase or ligase (XREF_SUPPLEMENTARY)."
25633985