Figure 8. Ataxin-3 isoforms show differences on physiological as well as pathophysiological levels. We found that ataxin-3 isoforms have a different stability and degradation pathway as well as a differing enzymatic activity. Moreover, they show differences in their interaction networks. On the pathophysiological level, isoforms show differences in their aggregation kinetics, number of aggregates per cell, and aggregate size. The line type shows effect strength or amount, and the tachometer is an indicator of the kinetics (10 o'clock/green, slow; 12 o'clock/yellow, moderate; 2 o'clock/red, fast).

Our study provides novel insights into the mechanisms of action of divalproex sodium as a possible treatment for SCA3, beyond the known regulation of transcription.