Fig. 1.

Simplified diagram of distal nephron sodium (Na)-transporting cell [distal convoluted tubule (DCT) cell and collecting duct principle cell (PC)]. The predominant apical Na transporter in the DCT cell is the thiazide-sensitive sodium chloride cotransporter (NCC) and in the collecting duct PC amiloride-sensitive epithelial sodium channel (ENaC), but some overlap exists between the cell types. Both are shown in the same cell in the diagram. For clarity, other transporters such as the potassium (K) channels are omitted. A aldosterone, C cortisol, GR peripheral tissue glucocorticoid receptor. Activation and suppression are indicated by circled + and circled − , respectively. Location of mutated proteins is indicated by circled numbers corresponding to the numbers in the text; red circles denote primary distal nephron disorders; blue circles denote adrenal and peripheral glucocorticoid receptor disorders