Accordingly, loss-of-function of enzymes involved in acylceramide metabolism result in cutaneous barrier abnormalities such as those found in human diseases and corresponding mouse models like ELOVL4-deficiency (MIM 614457) [32]–[34] or Chanarin-Dorfman syndrome (MIM 275630) [35], [36]. Here, ELOVL4 is linked to Dorfman-Chanarin disease.