However, osteoclast differentiation or osteoclast function is presumed to be suppressed in patients with OL-HED-ID and is presumed to be caused by IKBKG mutations for the following reasons: (i) NEMO is important for the RANKL-stimulated activation of NF-κB [54], (ii) osteoclasts are not formed in p50/p52 double-knockout mice [15,16], and (iii) myeloid cell-specific NEMO-deficient mice develop increased bone mass because of the inhibition of osteoclast formation [54]. Here, NFKB1 is linked to hypohidrotic ectodermal dysplasia.