VCP and amyotrophic lateral sclerosis: In addition, an ATP7A variant that results in the distal neuropathy phenotype was recently found to disrupt its interaction with p97/VCP, an ATPase involved in protein degradation which is mutated in motor neuron diseases such as amyotrophic lateral sclerosis (ALS), inclusion body myopathy with early -onset Paget disease and frontotemporal dementia (IBMPFD), and Charcot -Marie -Tooth disease type 2Y (Gonzalez et al., 2014; Johnson et al., 2010; Watts et al., 2007; Yi and Kaler, 2018).