Over-expression of Kir2.1 or several ATS-causing Kir2.1 mutants, including T75R and R218W mutations that interfere with trafficking of Kir2.1 (Tani et al., 2007), in X. laevis gives rise to craniofacial abnormalities of the eyes, jaw, and branchial arches (Adams et al., 2016), much like the phenotypes observed in mice and ATS patients. This evidence concerns the gene KCNJ2 and Andersen-Tawil syndrome.