IndraLab

Statements

STUB1 inhibits ATXN3. 7 / 7
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"CHIP has also been shown to degrade Ataxin-3 [ 59 ] ."
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"CHIP has also been shown to degrade Ataxin-3 [XREF_BIBR]."
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"Additionally, reduction in CHIP expression enhances expanded ataxin-3 toxicity in vivo."
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"Interestingly, when expanded ataxin-3 was present, CHIP levels were also reduced in the brains of MJD transgenic mice, raising the possibility that loss of one or both E3 partners may be a contributing factor in the pathogenesis of SCA3."
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"In contrast to ataxin-1, CHIP indeed promoted the degradation of polyQ expanded ataxin-3 (73Q) without causing its accumulation in the insoluble fraction, especially at the highest concentration."
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"In parallel, CHIP reduction markedly increases the level of ataxin-3 microaggregates, which partition in the soluble fraction of brain lysates yet are resistant to dissociation with denaturing detergent, and which precede the appearance of inclusions."
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"The dose dependent phenotype caused by CHIP reduction in Q71-B mice allowed us to determine whether CHIP reduction enhances ataxin-3 aggregation and to correlate biochemically detectable changes in aggregation to behavioral phenotype."
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