Abstract
EPITHELIAL Na CHANNELS (ENaC) are expressed in the aldosterone-sensitive distal nephron, where they serve as the final site of renal Na reabsorption and participate in the regulation of extracellular fluid volume and blood pressure. ENaC gain-offunction mutations are associated with hypertension, whereas loss-of-function mutations are associated with hypotension. Appreciation of the features that regulate ENaC expression and activity contributes to our understanding of the role of the kidney in the regulation of blood pressure. ENaC structure, assembly, and maturation. ENaC is assembled into a presumed trimeric structure (111) from three highly homologous subunits within the endoplasmic reticulum (ER), although higher ordered stoichiometries have also been proposed (3, 12, 21). The assembly appears to be inefficient given that only a limited fraction of newly synthesized subunits
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