Indeed, recent data implicate SGs and deregulated proteostasis in amyotrophic lateral sclerosis, frontotemporal lobar degeneration and multisystem proteinopathy, which are also associated with VCP mutations49, 50, 51, 52, 53, 54 and where protein aggregates that contain SG components accumulate. This evidence concerns the gene VCP and amyotrophic lateral sclerosis.