Figure 10.
Model showing the multiple factors contributing to the tunable dynamics of ataxin-1 NBs. Ataxin-1 can transit through three stages: diffuse nuclear protein, liquid droplet and then hydrogel, with multiple factors controlling these transitions. Initially, perturbations in ataxin-1 protein level or RNA/transcription, or by 1,6-hexanediol, can influence the transition from diffuse protein to dynamic liquid droplets that are visualized as distinct spherical ataxin-1 NB; fusion can result in larger NBs, but additional factors of time, altered RNA helicases by inhibitor RK-33 or siRNA, ATP depletion, or environmental stresses impact these ataxin-1 NBs to establish a less exchange dynamic and molecular fluctuation hydrogel state.