IndraLab

Statements

ATXN3 activates DNA Damage. 5 / 5
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"Previous studies have found a series of mitochondrial dysfunctions in the SCA3/MJD cell model ( Yu et al., 2009 ), such as decreased levels of antioxidant enzymes, increased mitochondrial DNA damage a[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
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"Recently it was shown that the expression of polyQ expanded ataxin-3 can lead to genomic DNA damage in neuroblasts through inactivation of polynucleotide kinase 3 '-phosphatase (PNKP) XREF_BIBR."
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"Enhanced accumulation of DNA damage has been observed in SCA3 patient post-mortem tissue and transgenic animal models of SCA3."
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"Additionally, a MJD and SCA3 in vitro model demonstrated that expanded ATX3 impairs the cell 's ability to respond to stress, alters antioxidant enzyme activities, and promotes mitochondrial DNA damage, which may lead to mitochondrial dysfunction."
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"Considering the data, increases in autophagy protein expression and stained cells with a lysosomotropic agent induced by HEME may play a significant role in mitigating the neurotoxic effects of mutant polyQ-expanded ataxin-3.Compared with controls, SCA3 patients exhibit lower levels of thiol protein and activity of antioxidant enzymes, such as SOD and GSH-PX, as well as higher oxidative stress and increased mitochondrial and nuclear DNA damage [29,30,31]."
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