Other examples are Wolfram syndrome, caused by mutations in WFS1 presenting with early-onset ataxia, cognitive deficits, and hearing loss, thus also including clinical hallmarks of the phenotype associated with DNAJC3 mutations (Khanim et al., 2001) and Wollcot-Rallison syndrome, an autosomal recessive disorder caused by mutations in eukaryotic translation initiation factor 2-alpha kinase 3 (E2AK3), characterized by insulin-dependent diabetes, growth retardation, and intellectual disability (Delepine et al., 2000). Here, EIF2AK3 is linked to Wolfram syndrome.