Intriguingly, Gly376Asp causes ALS, but Gly376Val was described to cause myopathy, with no evidence of neurodegeneration.21 The Trp385IlefsTer10 truncation is also associated with myopathy, specifically a rimmed vacuole myopathy, with TDP-43 aggregation observed in sarcomeres. Here, TARDBP is linked to amyotrophic lateral sclerosis.