IndraLab

Statements

ATXN3 inhibits DNA-templated transcription. 11 / 11
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"Since expanded ATX3 may disrupt normal gene transcription patterns, lithium may act as a protective factor by modulating gene expression."
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"Furthermore, microarray gene expression profiling in MJD and SCA3 transgenic mice revealed that expanded ATX3 may cause cerebellar dysfunction and ataxia by disrupting the normal pattern of gene transcription."
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"Notably, ATXN3 depletion significantly decreased global transcription, repair of transcribed genes, and error-free double-strand break repair of a 3 '-phosphate-containing terminally gapped, linearized reporter plasmid."
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"Ataxin-3 is thought to repress transcription via histone dependent chromatin remodeling [XREF_BIBR, XREF_BIBR], and huntingtin modulates the expression of NRSE controlled genes [XREF_BIBR]."
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"50 Interestingly, ATXN3 has been reported to repress transcription through recruitment of HDAC3 to target promoters 38 or by inhibiting a histone acetyltransferase."
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"Additionally, ataxin-3 also binds coactivators like CBP, p300, and CBP and p300 associated factor (PCAF) and represses the respective coactivator mediated transcription."
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"In the present study we use cell transfections, in vitro binding, co-immunoprecipitations, and reporter assays to show that the polyglutamine disease protein, ataxin-3, interacts with the major histone acetyltransferases cAMP-response-element binding protein (CREB)-binding protein, p300, and p300 and CREB binding protein associated factor and inhibits transcription by these coactivators."
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"Similar to the polyQ-expansions of sizes> = 34 in the polysome associated protein ATXN2 causing Spinocerebellar Ataxia Type 2 (SCA2), polyQ expansions of sizes> = 52 in the deubiquitinating transcript[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
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"ATXN3 depletion leads to increased global transcription."
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"ATX-3 deletion destabilizes p53, resulting in deficiency of p53 activity and functions, whereas ectopic expression of ATX-3 induces selective transcription and expression of p53 target genes and promotes p53 dependent apoptosis in both mammalian cells and the central nervous system of zebrafish."
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"The lack of ATXN3 leads to abnormalities in nuclear and nucleolar morphology , alters DNA replication timing and increases transcription ."
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