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Disorders of Steroid 11β-Hydroxylase Isozymes^*

Department of Pediatrics, Cornell University Medical College New York, New York 10021

Correspondence: Address requests for reprints to: Perrin C. White, M.D., Division of Pediatric Endocrinology, The University of Texas Southwestern Medical Center at Dallas, 5323 Harry Hines Boulevard, Dallas, Texas 75235.

Abstract

IT WAS first demonstrated in 1929 that extracts of adrenal glands could indefinitely sustain patients with Addison's disease (1). During the following decade a number of steroids were isolated from such extracts and their properties determined (2, 3). The actions of corticosteroids included amelioration of signs of stress and hyperglycemic (glucocorticoid) effects on the one hand and sodium retaining, potassium excreting (mineralocorticoid) effects on the other (4). Bioassays for these activities were complex. For example, in one widely used assay to test the ability of corticosteroids to ameliorate signs of stress, the gastrocnemius muscle of an adrenalectomized, anesthetized rat was electrically stimulated to lift a weight, and the total work performed before exhaustion was measured. Steroids with an oxygen atom at C11, such as corticosterone (compound B) and cortisone (compound E), were far more active in such assays than 11-deoxycorticosterone (5). Identification of cortisol (pregn-4-ene-11, 17, 21-triol-3, 20-dione) as the most important glucocorticoid hormone in humans came from studies in the late 1940s of patients with Cushing's syndrome or surgical stress, who were found to excrete large amounts of this steroid in the urine (6).

Footnotes

^* Supported by NIH Grants DK-37867 and DK-42169.

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