Consequently, loss of the crosslinking enzymes transglutaminase 1 and 5 which affect several CE proteins results in perturbation of CE’s and barrier function in mice [43] and have been associated with Acral peeling skin syndrome (missense mutation in TGM5) and lamellar ichthyosis (mutation in TGM1 resulting in deficiency) [44,45,46]. Here, TGM1 is linked to acral peeling skin syndrome.