FUS and proteostasis deficiencies: Importantly, by introducing multiple mutations in the RRM RNA-binding domain of R495X FUS, to reduce its RNA-binding ability (Daigle et al., 2013), they partially abrogated R495X-induced effects on mRNA translation, mitochondrial size, and neurotoxicity, uncovering a novel RNA-mediated pathway of FUS proteinopathy (Nakaya and Maragkakis, 2018).