These include gene products involved in essential processes of the control of cell growth (P63, ectodermal dysplasia), transcriptional regulation (NEMO/IKKγ, incontinenta pigmenti; PATCHED, nevoid basal cell carcinoma or Goltz-Gorlin’s syndrome), telomeric (congenital dyskeratosis), and non-telomeric genome maintenance (xeroderma pigmentosum). The gene discussed is IKBKG; the disease is ectodermal dysplasia syndrome.