IndraLab

Statements

ATXN3 activates Death. 14 / 14
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"The above results indicate that the expanded polyQ tract in ataxin3 could change the DNA methylation status of a variety of genes involved in various biological processes and, thus, cause neuronal death in the cerebellum and spinal cord due to alterations in the expression of these genes."
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"TUNEL staining was also performed to test the hypothesis that mutant polyglutamine ataxin-3 causes apoptotic neuronal death of pontine nuclei."
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"We found that ubiquitous expression of pathogenic Atxn3(Q80) with intact UbS1 leads to developmental death in pupal and pharate adult phases; no adults eclose successfully from their pupal cases (Figure 1C)."
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"Spinocerebellar ataxia type 3 (SCA3) is the most frequent inherited cerebellar ataxia in Europe, the US and Japan, leading to disability and death through motor complications."
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"In SCA3, a polyglutamine expansion in ATXN3 causes neuron loss in disease-vulnerable brain regions, resulting in progressive loss of coordination and ultimately death."
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"Ataxin-3, normally distributed in the cytoplasm, forms insoluble aggregates in the nuclei of neurons in MJD patients and is thought to cause cell dysfunction and death ."
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"It is caused by a CAG over repetition in ATXN3 gene, which translates into a mutated ataxin- 3 protein that accumulates in neurons, causing neuronal dysfunction and death."
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"These data supported an idea that, due to enhanced interaction to p53 and up-regulation of p53, polyQ expanded ATX-3 led to an increased p53 dependent neuronal cell death (including both early apoptotic and late apoptotic and necrotic manner)."
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"MJD leads to premature death and there is no therapy available."
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"Recent studies showed that polyglutamine-expanded huntingtin or ataxin-3 also induced caspase-3 activation and apoptotic neuronal death by releasing cytochrome- c and Smac from mitochondria ( [48,49] [MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
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"Taken together, this study provides evidence showing that the truncated ATXN3 accelerates the formation of aggregates, disrupts the dynamics of mitochondria, and leads to neuronal death in vitro and in vivo."
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"Spinocerebellar ataxia type 3 (SCA3) is caused by the expansion of a polyglutamine (polyQ) repeat in the protein ataxin-3 which is involved in susceptibility to mild oxidative stress induced neuronal death."
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"In the presence of adenosine A 2A receptor antagonist SCH58261, T1-11 or JMF1907 failed to prevent mutant ataxin-3-Q79-induced cerebellar transcriptional downregulation of SCA3 mice.Polyglutamine-expa[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
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"In addition, they found that expanded ataxin3 could activate the mitochondrial apoptotic pathway and induce neuronal death by regulating gene expression [XREF_BIBR]."
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