IndraLab

"Wild-type ATXN3 can be degraded by the proteasome, but whether other protein quality control pathways are involved in its turnover remains unknown."

"Ataxin-3 is degraded by the proteasome."

"Finally, pulse-chase labeling reveals that ataxin-3 is degraded by the proteasome, with expanded ataxin-3 being as efficiently degraded as normal ataxin-3."

"However, proteasome inhibition triggered polyQ expanded ATXN3 aggregation, a process that could be linked with the global proteostasis collapse induced by this treatment."

"In vitro studies revealed that inactive ataxin-3 was more slowly degraded by the proteasome and that this degradation occurred independent of ubiquitination."

"For ataxin-3 to be degraded by the proteasome, it needs to come into contact with this cellular machinery."

"The neuroprotective role of HSJ1 has been demonstrated in different disease models : it suppresses the aggregation of polyglutamine expanded proteins, significantly enhancing mutant huntingtin solubility in Huntington disease in cells and in mice, and promoting misfolded protein targeting to the ubiquitin-proteasome system; HSJ1a cooperates with Hsp70 to promote proteasome degradation of ataxin-3, a protein responsible for spinocerebellar ataxia type 3 (SCA3); HSJ1a prevented the aggregation of the misfolded C289G Parkin, a Parkinson disease associated ubiquitin protein ligase mutant, and restored its function in mitophagy."

"p45, an ATPase subunit of the 19S proteasome, interacts with ataxin-3 in vitro and stimulates the degradation of ataxin-3 in an in vitro reconstituted degradation assay system."