In 1985, several years before TG2 was identified as autoantigen in CeD [33], and as the enzyme involved in the creation of deamidated gluten T-cell epitopes [2, 3], Bruce et al. reported that TG2 has preference for gliadin as substrate and moreover, that the catalytic transglutaminase activity is increased in jejunal biopsies of patients with UCeD compared to controls subjects and patients with inflammatory bowel disease [34]. Here, TGM2 is linked to cranioectodermal dysplasia.