Protein Disulphide Isomerase (PDI) Is Protective against Amyotrophic Lateral Sclerosis (ALS)-Related Mutant Fused in Sarcoma (FUS) in In Vitro Models

Scientists demonstrated that protein disulphide isomerase (PDI) was protective against mutant Fused in Sarcoma (FUS). In neuronal cell line and primary cultures, PDI restored defects in nuclear import, prevented the formation of mutant FUS inclusions, inhibited Golgi fragmentation, ER stress, ER-Golgi transport defects, and apoptosis.