SUMO-1 promotes degradation of the polyglutamine disease protein ataxin-3

Polyglutamine diseases are caused by the expansion of polyQ repeats in the respective gene products. Intracellular accumulation of disease proteins is a hallmark of polyQ diseases; however, whether the increased aggregation of polyQ protein is the cause of diseases is still controversial. Efforts have been made to clarify the relationship between protein aggregation and pathogenesis of polyQ disease. Especially, the roles of post translational modifications garner attention because of their roles in regulating protein stability and aggregation.