Response to an llβ-Hydroxylase Inhibitor (SU-4885) in Males with Adrenal Hyperplasia and in Their Parents

Cleveland, William W.; Nikezić, M; Migeon, Claude J.

doi:10.1210/jcem-22-3-281

Cited by 33 publications

(9 citation statements)

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“…Administration of this compound to normal man results in the accumulation of 17,21-dihydroxy-A4-pregnene-3,20-dione and other ll-deoxysteroids in the urine. Migeon and colleagues (209) administered SU 4885 to patients with the adrenogenital syndrome and defective C-21 hydroxylation. As antici pated, C-11 and C-21 dideoxysteroid metabolites accumulated in the urine.…”

Section: Genetic Defects In Steroid Metabolismmentioning

confidence: 99%

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: 99%

Enzymatic Mechanisms in Steroid Biochemistry

Talalay¹

1965

Annu. Rev. Biochem.

105

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“…Since no clinical abnormality is presented by heterozygous subjects, numerous biological in¬ vestigations have been undertaken in order to detect any minor impairments of 21-hydroxylation which they may have. One manifestation is the presence of excessive urinary pregnanetriol following ACTH (Childs et al 1956) or metyrapone tests (Cleveland et al 1962). However, the results of such tests have shown considerable overlap between heterozygous and control sub¬ jects.…”

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confidence: 99%

Detection of heterozygous carriers for 21-hydroxylase deficiency by plasma 21-deoxycortisol measurement

Gourmelen

Gueux

Trung

et al. 1987

Acta Endocrinologica

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Using a highly specific radioimmunoassay recently described, plasma 21-deoxycortisol levels were measured in 55 heterozygous carriers of 21-hydroxylase deficiency (as demonstrated by HLA typing).Mean baseline 21-deoxycortisol levels were above the normal range, but there was a 38% overlap with control values. In contrast to 17-hydroxyprogesterone levels, which in 71% of the subjects remained within the normal range one hour after ACTH stimulation, 21\ x=r eq-\ deoxycortisol levels increased over stimulated control levels in all but two heterozygous carriers. No differences as to the levels were observed between heterozygous carriers for the classic and the late-onset forms. Plasma 21-deoxycortisol measurement appears to be a valid tool in the biological detection of heterozygosity for 21-hydroxylase deficiency and its implications in genetic counselling.

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“…There is no true difference in the sex ratio (Childs et al, 1956;Prader et al, 1962;Prader and Anders, 1962). Up to the present it has not been possible to detect the heterozygous carrier of the gene (Childs et al, 1956;Cleveland and Migeon, 1960). Prader (1958) has estimated an incidence of the gene of 1: 37 5 in the general population in Switzerland, which leads to one adrenogenital syndrome patient among every 5,000 births in Switzerland.…”

Section: Iv-adrenocortical Hypofunctionmentioning

confidence: 99%

The adrenal cortex in childhood. 2. Pathological aspects.

Visser¹

1966

Archives of Disease in Childhood

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Response to an llβ-Hydroxylase Inhibitor (SU-4885) in Males with Adrenal Hyperplasia and in Their Parents

Cited by 33 publications

References 0 publications

Enzymatic Mechanisms in Steroid Biochemistry

Enzymatic Mechanisms in Steroid Biochemistry

Detection of heterozygous carriers for 21-hydroxylase deficiency by plasma 21-deoxycortisol measurement

The adrenal cortex in childhood. 2. Pathological aspects.

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