Disorders of the epithelial sodium channel: Insights into the regulation of extracellular volume and blood pressure  Principal discussant: John B. Stokes  Kidney International  Volume 56, Issue 6, Pages 2318-2333 (December 1999) DOI: 10.1046/j.1523-1755.1999.00803.x Copyright © 1999 International Society of Nephrology Terms and Conditions

Figure 1 Schematic of the action of aldosterone on the principal cell of the cortical collecting duct. The major action is to increase the permeability of the Na+ channel on the apical membrane; this channel is inhibited by amiloride and triamterene. MR, mineralcorticoid receptor. Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions

Figure 2 (A) Diagram of a single subunit of ENaC. The general features, two membrane-spanning domains, a large extracellular loop, and intracellular amino- and carboxy-termini, are typical of all members of this family. (B) Schematic drawing indicating arrangement of α-, β-, and γ-ENaC into one Na+ channel. The actual channel complex consists of more than the number of subunits shown here. Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions

Figure 3 The members of the ENaC/DEG family. Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions

Figure 4 Mutations in ENaC subunits causing pseudohypoaldosteronism. Triangles indicate premature stop codons or nonsense mutations. Dots indicate missense mutations causing one amino acid substitution. Further details on these mutations are shown in Table 2. Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions

Figure 5 Mutations in ENaC subunits causing Liddle's syndrome. Short lines represent premature stop codons or nonsense mutations. Dots indicate missense mutations causing one amino acid substitution. Further details on these mutations are shown in Table 4. Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions

Figure 6 (A) Interaction of one ENaC subunit with Nedd4, a protein involved in regulating surface expression. (B) Endoctyosis of a single ENaC subunit as a result of its interaction with Nedd4 and its ubiquitination (represented by a circle). Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions

Kidney International 1999 56, 2318-2333DOI: (10. 1046/j. 1523-1755 Kidney International 1999 56, 2318-2333DOI: (10.1046/j.1523-1755.1999.00803.x) Copyright © 1999 International Society of Nephrology Terms and Conditions