There is also another situation with similar presentation, i.e. familial hyperkalemic hypertension (also known as pseudohypoaldosteronism type 2 or Gordon syndrome), which is associated with sodium and potassium retention due to defects in genes encoding serine-threonine kinases (WNK1 and WNK4) or ubiquitinating enzymes (CUL3 and KLCH3) expressed in the distal nephron and altering expression of the sodium-potassium co-transporter (22–24). The gene discussed is MARK2; the disease is pseudohypoaldosteronism type 2.