In addition, the RIPK4 p.Ile121Asn missense mutation, which has been identified in Bartsocas-Papas syndrome, inhibits the kinase activity of RIPK4, thereby abolishing RIPK4-mediated IRF6 and NF-κB activation (14, 18). Here, RIPK4 is linked to Bartsocas-Papas syndrome 1.