TGM2 and cranioectodermal dysplasia: The functional relevance of this amplification loop was indeed confirmed in the clinical study in which the inhibition of TG2 activity by ZED1227 in individuals with CeD significantly inhibited both the (epithelial) IFNγ response (Fig. 3b) and TGM2 expression (Fig. 3d), resulting in protection from villous atrophy and intraepithelial lymphocytosis (Fig. 2d).