IndraLab

Statements

Mutated ATXN3 activates ATXN3. 11 / 11
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"Mutant ATXN3 activates the pro apoptotic p53 pathway by activating ATM in SCA3."
25590633
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"Recent studies have reported that depletion of the mutant ATXN3 allele in a SCA3 transgenic mouse brains rescues the molecular phenotypes of SCA3 supporting the hypothesis that mutant ATXN3 elicits toxicity and neuronal dysfunction in SCA3 [XREF_BIBR]."
25590633
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"Mutant ATXN3 induces genomic DNA damage in SCA3."
25590633
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"Nonetheless, a significant understanding of the disease etiology of SCA3, the molecular mechanism by which the polyQ expansions in the mutant ATXN3 induce neurodegeneration in SCA3 has remained elusive."
25590633
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"As expected, the antibody combination 1H9 and MW1 shows a mutant ataxin-3 specific signal in SCA3 transgenic mice, whereas the antibodies 2B7 and MW1 specifically bind to mutant huntingtin in transgenic Huntington mice (XREF_FIG)."
23626768
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"Machado-Joseph disease (MJD), also called spinocerebellar ataxia type 3, is caused by mutant ataxin-3 with a polyglutamine expansion."
16389311
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"Mutant ATXN3 activates the DNA damage response pathway in SCA3."
25590633
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"While many studies indicate that SCA3 disease is predominantly caused by the mutant ATXN3 protein, SCA3 rCAG exp RNA has also been implicated in SCA3 pathogenesis."
25201111
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"Autophagy also has a role in clearance of other polyglutamine expanded proteins, including mutant ataxin-3 that is causing the spinocerebellar ataxia type 3 (SCA3) [XREF_BIBR]."
22518139
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"Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease is an autosomal dominant neurodegenerative disease and is caused by the mutation of ATXN3 gene that encodes ataxin-3 (Kawaguchi et al., 1[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
25139423
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"To evaluate allele specificity by LNA8-17Dz9, we co-transfected the DNAzyme with either ATXN3-Q28 or ATXN3-Q84 (mutant ATXN3 causes SCA3) in HEK293 cells."
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