Mutations in p97 are causative of a multitude of muscular and neural degenerative diseases [41], including amyotrophic lateral sclerosis (ALS), Charcot-Marie-Tooth type 2Y, inclusion body myopathy associated with Paget’s disease of the bone and front-temporal dementia (IBMPFD), and multisystem proteinopathy [42,43]. Here, VCP is linked to amyotrophic lateral sclerosis.