Moreover, the NEMOUBAN point mutation D311N or the deletion of the C-terminal zinc finger (E391X: Δ391–417), two patient derived mutations that cause anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID), abolished TNFα-induced NF-κB signaling, but only mildly affected IL-1β stimulated NF-κB activation (Fig. 1d,e). Here, TNF is linked to hypohidrotic ectodermal dysplasia.