Comparative analyses of disease-linked missense mutations in the RNA exosome modeled in budding yeast reveal distinct functional consequences in translation

Translation fidelity defects in the RNA exosomopathy mutant yeast models compared to wild-type control cells. Dual-luciferase reporter assays were used to measure translation fidelity. The expression of firefly and Renilla luciferase was measured in rrp4-G226D (A), rrp40-W195R (B), and rrp46-L191H (C) mutant cells, compared to the corresponding wild-type control cells. The ratio of firefly luciferase to Renilla luciferase was normalized to control plasmids. Four to 12 biological replicates were analyzed.