Polyglutamine repeat expansion in ataxin-3 causes neurodegeneration in spinocerebellar ataxia type 3, and reducing the levels of the mutant protein improves pathology. Here the authors show that ataxin-3 is degraded through an ubiquitination-independent mechanism that is impeded by protein interactions with proteasome-associated factors.
- Jessica R. Blount
- Wei-Ling Tsou
- Sokol V. Todi
