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11ß-Hydroxysteroid Dehydrogenase and the Syndrome of Apparent Mineralocorticoid Excess¹

Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas Texas 75235-9063

I. Introduction

II. Biochemistry of Cortisol Metabolism

III. Mineralocorticoid Receptor Function

A. Actions of aldosterone

B. Structure and function of the mineralocorticoid receptor

C. Hypothesis: 11-HSD protects the mineralocorticoid receptor

IV. Loss of Function of 11-HSD

A. Syndrome of apparent mineralocorticoid excess (AME)

B. Licorice intoxication

C. Ectopic ACTH syndrome

D. Essential hypertension

E. Related conditions

V. Functional Roles of 11-HSD

A. Liver

B. Kidney and other mineralocorticoid target tissues

C. Brain

D. Circulatory system

E. Skin

F. Ovary

G. Placenta

H. Other fetal tissues

VI. The Type I (Liver) Isozyme of 11-HSD

A. Terminology

B. Biochemistry

C. Molecular biology

D. Expression

E. Lack of involvement in the syndromes of AME or 11-reductase deficiency

VII. The Type 2 (Kidney) Isozyme of 11-HSD

A. Biochemistry

B. Molecular biology

C. Expression

D. Mutations in HSD11B2 are detected in all patients with AME

VIII. Summary\.