IndraLab

Statements


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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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"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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"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."