Figure 8.

Neurotoxicity of Mutant FUS in C. elegans and Reduced New Protein Synthesis in Xenopus Neurons Are Unlikely to be Due to Activation of the UPR due to Accumulation of Misfolded FUS

(A) In vivo stress granule density measured by counting pab-l-positive granules in C. elegans under basal conditions and after heat shock were not different between control (empty vector); FUS(WT) and mutant FUS animals, indicating that mutant FUS animals were not under greater unfolded protein stress.

(B) Thapsigargin treatment induced UPR and reduced new protein synthesis in Xenopus neurons expressing FUS(WT). PERK1 inhibitors rescued reduced protein synthesis in thapsigargin-treated neurons expressing FUS(WT), but not mutant FUS-expressing neurons.

Error bars are SEM.