While mutations in Vps54 have not been directly linked to human disease, the wobbler mouse has been used by many researchers as a model for the sporadic form of ALS because of striking similarities to ALS pathology (Ikeda et al., 1998; Dennis and Citron, 2009; Nieto-Gonzalez et al., 2011; Moser et al., 2013; Dahlke et al., 2015; Schmitt-John, 2015; Klatt et al., 2019; Rohm et al., 2019; Cihankaya et al., 2021; De Nicola et al., 2021). This evidence concerns the gene VPS54 and amyotrophic lateral sclerosis.