The importance of WNK signaling in potassium metabolism is evidenced by familial hyperkalemic hypertension, a Mendelian syndrome caused by overactivation of the DCT-expressed WNK signaling pathway, resulting in NCC hyperphosphorylation, salt-sensitive hypertension, and hyperkalemia that is cured with thiazide diuretics (7, 8). The gene discussed is SLC12A3; the disease is Hyperkalemia.