IndraLab

Statements



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"ATX-3 Promotes p53 Dependent Apoptosis in Cells and in Zebrafish."

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"SUMOylation did not influence the subcellular localization, ubiquitination or aggregates formation of mutant-type ataxin-3, but partially increased its stability and the apoptosis rate of the cells."

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"Deletion of ATXN3 resulted in destabilization of p53, whereas ectopic expression of ATXN3 induced expression of p53 target genes and promoted p53-dependent apoptosis."

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"In addition, ATXN3 SUMOylation by SUMO-1 on site K166 also increases apoptosis in SCA3; therefore, SUMOylation by SUMO-1 might stimulate SCA3 pathogenesis through both effects described above."

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"As P53 has known functions in cycle arrest and apoptosis, this indicates that expression of atxn3 polyQ repeats induces selective transcription and expression of p53 target genes and promotes p53 dependent apoptosis in the CNS of zebrafish [XREF_BIBR]."
| PMC

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"Using the zebrafish model system, we further examined whether ATX-3 induces p53 dependent apoptosis in vivo."

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"These workers have also shown that overexpression of the MJD protein with expanded polyglutamine repeats leads to apoptosis, both in vitro and in vivo."

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"In conclusion, we demonstrated that lithium carbonate and CoQ10 reduced apoptosis induced by expanded ATX3 (Q84)."

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"SUMOylation did not influence the subcellular localization, ubiquitination or aggregates formation of mutant-type ataxin-3, but partially increased its stability and the cell apoptosis."

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"When the FL ATX-3 mRNA was injected into WT but not the p53 mutant zebrafish embryos, significantly more apoptotic cells were observed in TUNEL staining assays, indicating that ATX-3 caused p53 dependent apoptosis in vivo."

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"Flow cytometry analysis using Annexin V-FITC and propidium iodide (PI) staining in HCT116 cells showed that knockdown of ATX-3 led to a decrease of camptothecin (CPT)-induced apoptosis, while ectopic expression of ATX-3 but not the catalytic inactive mutant ATX-3-C14A resulted in a significant increase of apoptosis in HCT116 p53 +/+ but not HCT116 p53 -/- cells (XREF_FIG), indicating that ATX-3 promoted p53 mediated apoptosis, which required its deubiquitinating enzymatic activity."

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"In a SCA 3 cell model, the expression of a fragment of ataxin-3 containing an elongated polyQ stretch induced apoptosis and cell death as well as a severe ataxia in a mouse model, showing a more rapid manifestation of a SCA 3-reminiscent phenotype when compared to mice expressing full length mutant ataxin-3 [XREF_BIBR]."