While the kinase function of IKKα is dispensable for keratinocyte differentiation (Hu et al., 2001), RIPK4 phosphorylates IRF6 to drive nuclear translocation and regulate its transactivator activity; mutations in the kinase domain of RIPK4 that underlie Bartsocas Papas syndrome disrupt this activity (De Groote et al., 2015; Kwa et al., 2014). This evidence concerns the gene CHUK and Bartsocas-Papas syndrome 1.