IKBKG and immune system disorder: NEMO is another protein involved in the pathogeneticcascade.Due to the fact that NF-κB also controls the immuneresponse and apoptosis, the clinical manifestations ofmutations in the NEMO gene are not only limited to damageto ectodermal structures, but also include immune system disorders,with the development, in particular, of anhidrotic ectodermaldysplasia with immunodeficiency 1 (OMIM 300291)(Smahi et al., 2002).