The first evidence linking WNK kinases to the CCCs was the discovery of mutations in the genes WNK1 and WNK4 in Familial Hyperkalemic Hypertension (FHHt), also known as Pseudohypoaldosteronism type 2 (PHAII), or Gordon syndrome (Wilson et al., 2001). Here, WNK4 is linked to pseudohypoaldosteronism type 2.