The critical role of WNKs in regulation of renal Na+ and K+ handling has been culminated by the seminal discovery that patients with pseudohypoaldosteronism type II (PHAII), also known as Gordon’s syndrome exhibit a rare combination of hypertension and hyperkalemia due to gain-of-function mutations in WNK1 and WNK4, which causes defective degradation of these kinases [23,24,25]. This evidence concerns the gene WNK1 and pseudohypoaldosteronism type 2.