NEMO (NF-κB essential modifier)/IKKγ (IκB kinase-γ) is required for the activation of the IκB
kinase complex (IKK) by inflammatory stimuli such as tumor necrosis factor (TNF-α). Here we
show that TNF-α stimulates the ubiquitination of NEMO in a manner that does not appear to
target it for degradation and that is impaired by mutations in the NEMO zinc finger. Mutations
of the zinc finger are found in patients with hypohidrotic ectodermal dysplasia with
immunodeficiency (HED-ID) and lead to the impairment of TNF-α-stimulated IKK …