Production of Congenital Adrenal Cortical Hyperplasia, Hypospadias, And Clitoral Hypertrophy (Adrenogenital Syndrome) in Rats by Inactivation of 3 -Hydroxysteroid Dehydrogenase
| Autor: | Allen S. Goldman, William C. Yakovac, Alfred M. Bongiovanni |
|---|---|
| Rok vydání: | 1966 |
| Předmět: |
Male
medicine.medical_specialty Congenital adrenal cortical hyperplasia General Biochemistry Genetics and Molecular Biology Internal medicine Adrenal Glands Testis medicine Animals Clitoral hypertrophy Adrenal Cortical Hyperplasia Fetus Adrenal Hyperplasia Congenital Histocytochemistry business.industry Anogenital distance Hydroxysteroid Dehydrogenases Models Theoretical medicine.disease Enzymes Rats Endocrinology Hypospadias Adrenogenital syndrome Gestation Steroids business Metabolism Inborn Errors |
| Zdroj: | Experimental Biology and Medicine. 121:757-766 |
| ISSN: | 1535-3699 1535-3702 |
| DOI: | 10.3181/00379727-121-30879 |
| Popis: | SummaryVarying daily doses of a steroidal inhibitor of 3β-hydroxysteroid dehydrogenase (2α-cyano-4,4,17α-trimethylandrost-5-en-17β-ol-3-one), were administered to pregnant rats on the 15 th to 20th days of gestation to determine whether this inhibitor could produce the biological manifestations of the human disease associated with deficient activity of this enzyme. A single dose of the inhibitor was also administered to pregnant rats on various days to determine the critical periods for production of these manifestations. Infants with this disorder have hyperplastic adrenal cortices deficient in activity of the 3β-enzyme, incomplete masculine development in males resulting in hypospadias with shortening of the anogenital distance, and clitoral hypertrophy without urethral orificial displacement in females. The experimental fetuses had adrenal cortical hyperplasia (average of 5.9 ± 1.32 mg against 2.2 ± 0.26 mg controls), deficient histochemical activity of the 3β-enzyme in the adrenals and testes, increas... |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|