Patients with mutations in HSD11B2 present with the syndrome of apparent mineralocorticoid excess whereby, as a consequence of the inability to clear cortisol locally (principally within the kidney), it is able to bind and activate the MR for which it shares the same affinity as aldosterone, resulting in severe hypertension with life-threatening hypokalemia (500). Here, NR3C2 is linked to Hypokalemia.