Both illnesses produce arterial hypotension accompanied by hypokalemic metabolic alkalosis and hypercalcemia due to poor NCC activity; however, when elevated NCC activity occurs, it leads to the development of a sodium-dependent form of hypertension known as Familial Hyperkalemic Hypertension or Pseudohypoaldosteronism type II, this disease is caused by mutations in the WNK1 and WNK4 kinases (Wilson et al., 2001) or the KLHL3 and Cul3 ubiquitin ligase proteins (Boyden et al., 2012; Louis-Dit-Picard et al., 2012). The gene discussed is CUL3; the disease is pseudohypoaldosteronism type 2.