They found significantly increased TDP-43 and pTDP-43 both in axons and in Schwann’s cells, and TDP-43 aggregates in ALS and IBM patient motor nerves even without axonal degeneration, pointing out that TDP-43 aggregation is likely an early event in the pathogenesis of motor nerve degeneration [121]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.