In Vitro Hydroxylation of Steroids by Whole Adrenal Homogenates of Beef, Normal Man, and Patients with the Adrenogenital Syndrome1

Bongiovanni, Alfred M.

doi:10.1172/jci103723

Cited by 48 publications

(10 citation statements)

References 24 publications

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“…The identification of this metabolic block was originally based on the finding of large quantities of 21-methyl steroids in the urine of such patients (200,201,203). More recently, Bongiovanni (204) has found a relative de ficiency of C-21 hydroxylase in two cases of this disease. The possibility that the C-21 methyl steroids arise by loss of the C-21 hydroxyl groups has been ruled out by Fukushima & Gallagher (205) who showed that only a negligible proportion of the radioactivity of administered cortisol-4-04 was recovered as 21-deoxysteroids in the urine of a patient with the adrenogenital syndrome.…”

Section: Genetic Defects In Steroid Metabolismmentioning

confidence: 89%

Enzymatic Mechanisms in Steroid Biochemistry

Talalay¹

1965

Annu. Rev. Biochem.

105

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This selective review of the present status of steroid biochemistry is con fined largely to consideration of the enzymatic basis of the transformations of steroids. This subject was comprehensively reviewed in 1957 (1). The in tervening years have witnessed considerable progress toward a better under standing of the manner of formation of the truly formidable number and variety of steroids from animal, plant, and microbial sources.Although this review is oriented toward discussion of the effects of en zymes on steroids, there is intense interest in the effects of steroids on enzyme systems and the possible relation of these phenomena to the molecular basis of steroid hormone action. Progress in the understanding of the details of the biosynthesis of both proteins and nucleic acids has made it possible to show that the regulatory influences of a number of hormones may find a common . basis in the control of the synthesis of various specific prot�ins. This control appears to be exerted at least in part on the process of the transcription of genetic information which directs protein synthesis. Many new studies have appeared in this area since the review by Tomkins & Maxwell (2), and excellent comprehensive discussions of this subject have been provided by Williams-Ashman (3, 4, 5), and by the book Actions of Hormones on Molecular Processes, edited by Litwack & Kritchevsky (6). These studies have not yet revealed the precise manner in which steroids modulate protein synthesis.However, it is now abundantly clear that steroid hormones may affect directly the activities of a number of relatively well-defined enzymatic pro cesses in two quite distinct ways: (a) certain steroids can participate in a catalytic manner in hydrogen transport reactions-the chemistry and pos sible physiological significance of these reactions has been reviewed in detail (7,8,9) ; (b) steroids may affect the activities of a number of enzymes in an "allosteric" way (2,5,7,9) , in accordance with the very imaginative pro posal of Monod, Changeux & Jacob (10).It has not been possible to cover all aspects of the enzymology of steroid transformations. Especially to be regretted are omissions of the discussion of contributions to sterol biosynthesis and the discovery of inhibitors which ap pear specifically to interrupt certain stages in the formation of cholesterol 1 The survey of literature pertaining to this review was completed in Decembe r 1964. 2 Studies from the author's laboratory were supported in part by grants from the National Institutes of Health of the United States Public Health Service. 347 Annu. Rev. Biochem. 1965.34:347-380. Downloaded from www.annualreviews.org Access provided by McMaster University on 02/05/15. For personal use only. Quick links to online content Further ANNUAL REVIEWS

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Section: Genetic Defects In Steroid Metabolismmentioning

confidence: 89%

Enzymatic Mechanisms in Steroid Biochemistry

Talalay¹

1965

Annu. Rev. Biochem.

105

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“…A deficiency of 21-hydroxylation has been reported in both the nonsalt-losing and the salt-losing forms of congenital adrenal hyperplasia (1)(2)(3)(4). Cortisol and aldosterone, the two biologically significant steroids secreted by the human adrenal cortex, require 21-hydroxylation for their biosynthesis.…”

Section: Discussionmentioning

confidence: 99%

Aldosterone Secretion Rate in Congenital Adrenal Hyperplasia. A Discussion of the Theories on the Pathogenesis of the Salt-losing Form of the Syndrome*

Kowarski¹,

Finkelstein²,

Spaulding³

et al. 1965

J. Clin. Invest.

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“…Since overproduction of pregnanetriol 1 and of pregnanetriolone (1) has been demonstrated in this disease, it has been assumed that the block in steroidogenesis -involves the 21-hydroxylation of 17a-hydroxyprogesterone (2). A defect in 21-hydroxylation of 17a-hydroxyprogesterone has been shown in vitro for two patients with the "salt-losing" form of congenital adrenal hyperplasia (3).…”

Section: Introductionmentioning

confidence: 99%

Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

Bartter¹,

Henkin²,

Bryan³

1968

J. Clin. Invest.

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A B S T R A C T Patients with the "non-salt-losing" form of the adrenogenital syndrome were studied before and after suppression of adrenal cortical activity with carbohydrate-active steroids. The response of aldosterone secretion to sodium deprivation was measured; in some patients response to adrenocorticotropic hormone (ACTH) was measured as well.The aldosterone secretion was normal and responded normally to sodium deprivation in all patients studied during suppression with carbohydrate-active steroids. This finding suggests that 21-hydroxylation of progesterone is normal in this syndrome.The sole abnormality in the production of aldosterone in these patients was found to be excessive secretion of aldosterone while they were not receiving suppressive doses of carbohydrate-active steroids. This finding strongly supports the view that the biogenetic pathways through which aldosterone is produced from progesterone are intact in this syndrome.No patient showed hypertension or hypokalemic alkalosis despite very high aldosterone secretion rates. This observation suggests that the hyperaldosteronism is secondary to a tendency to sodium loss in the patient whose ACTH production is not suppressed.

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In Vitro Hydroxylation of Steroids by Whole Adrenal Homogenates of Beef, Normal Man, and Patients with the Adrenogenital Syndrome1

Cited by 48 publications

References 24 publications

Enzymatic Mechanisms in Steroid Biochemistry

Enzymatic Mechanisms in Steroid Biochemistry

Aldosterone Secretion Rate in Congenital Adrenal Hyperplasia. A Discussion of the Theories on the Pathogenesis of the Salt-losing Form of the Syndrome*

Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

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