SOD1 and amyotrophic lateral sclerosis: Importantly, dysregulation of ER-Golgi trafficking preceded protein aggregation, ER stress, Golgi fragmentation and axon degeneration in NSC-34 cells expressing mutant SOD1 [78], and dysregulated ER-Golgi trafficking was observed as an early event in embryonic cortical and motor neurons in a mouse model of ALS [80].