Figure 1.

Amyotrophic lateral sclerosis (ALS)-linked proteins with yeast prion-like domains have been modeled in yeast. The prion domains of naturally occurring yeast prion proteins have similar size and composition, but are not homologous to the prion-like domains of ALS-linked proteins. The tools for studying yeast prions have been applied to ectopic expression of human disease proteins to evaluate aggregation and toxicity mechanisms. RRM, RNA recognition motif; RGG, arginine-glycine-glycine rich domain; ZnF, zinc finger domain.