Although the specific function of IKKγ in osteoclasts has not been fully elucidated, mutations in the IKKγ gene have been reported in patients with X-linked recessive ectodermal dysplasia with immunodeficiency (EDA-ID) as well as in those with osteosclerosis accompanied by lymphedema (OL-EDA-ID)72. Here, IKBKG is linked to hypohidrotic ectodermal dysplasia.