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Although in vitro studies with artificial substrates failed to show an impact of the expansion of the polyQ segment of ATXN3 on its DUB activity [37, 73, 74], the insolubility and immobile nature of the inclusions in vivo [75] may make mutant ATXN3 less prompt to locate at the sites at which editing of Ub chains is needed

All references to published data, proposed mechanisms, or preclinical observations are included solely for scientific and educational discussion

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