In dogs (Farias et al., 2011; Wohlke et al., 2011) and mice (Schultheis et al., 2013), loss of ATP13A2 elicits neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disorder characterized by the accumulation of autofluorescent lipopigment. This evidence concerns the gene ATP13A2 and infantile neuronal ceroid lipofuscinosis.