Wolframin, along with the structurally-unrelated and more widely phyletically distributed (Figure 2A) Wolfram syndrome 2 (WFS2) protein, is implicated in Wolfram syndrome (Inoue et al., 1998; Strom et al., 1998; Amr et al., 2007; Urano, 2016). This evidence concerns the gene WFS1 and Wolfram syndrome.