Teulon and colleagues speculated that the hypokalemia is most likely to have a cause outside the DCT and that the low urine pH and low renal ammonium excretion are indicative of a functional defect in the proximal tubule, where Kir5.1 is also expressed (Paulais et al., 2011). The gene discussed is KCNJ16; the disease is Hypokalemia.