In humans, gain-of-function mutations in WNK1 and WNK4 result in Familial Hyperkalemia and Hypertension (FHHt; sometimes referred to as pseudohypoaldosteronism type II (PHAII) or Gordon Syndrome), a monogenic form of secondary hypertension characterized by hypertension and hyperkalemia [59, 93, 183]. The gene discussed is WNK1; the disease is pseudohypoaldosteronism type 2.