Importantly, a recent genomic analysis has shown that mutations in MAP3K7 (encoding TAK1) or TAB2 found in Frontometaphyseal dysplasia (FMD)—one of the otopalatodigital syndrome spectrum disorders—cause increased TAK1 autophosphorylation at Thr187 and activation of MAP kinase and NF-κB pathway, suggesting that excessive activation of TAK1 accounts for a human developmental disorder [120]. Here, NFKB1 is linked to otopalatodigital syndrome spectrum disorder.