Mutations in DnaJC5, cause adult onset, neuronal ceroid lipofusinosis (ANCL), a neurodegenerative disorder characterized by lysosomal accumulation of autofluorescent oxidized lipid and protein waste, called lipofuscin (Benitez et al., 2011; Nosková et al., 2011; Velinov et al., 2012). Here, DNAJC5 is linked to adult neuronal ceroid lipofuscinosis.