9 results match your criteria C-17 Hydroxylase Deficiency

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Other conceivable renditions of some of the oxidative processes used in the biosynthesis of steroid hormones.

J Steroid Biochem Mol Biol 2006 Sep 17;101(1):31-41. Epub 2006 Aug 17.

Department of Obstetrics and Gynecology, Institute for Health Sciences, St. Luke's-Roosevelt Hospital Center, 432 West 58 Street, New York, NY 10019, USA.

The generally accepted version (GAV) of the chemical processes by which the steroid hormones are biosynthesized cannot be considered to be an inerrant description of in vivo processes. Customarily this version is derived by piecing together the results obtained from several independent artificial in vitro incubation experiments. Extrapolation of such results from in vitro to in vivo requires untested assumptions which introduce varying degrees of uncertainty. Read More

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September 2006

Ablation of the otcC gene encoding a post-polyketide hydroxylase from the oxytetracyline biosynthetic pathway in Streptomyces rimosus results in novel polyketides with altered chain length.

J Biol Chem 2005 Nov 7;280(45):37455-60. Epub 2005 Sep 7.

Department of Bioscience, University of Strathclyde, Glasgow, Scotland, UK.

Oxytetracycline (OTC) is a 19-carbon polyketide antibiotic made by Streptomyces rimosus. The otcC gene encodes an anhydrotetracycline oxygenase that catalyzes a hydroxylation of the anthracycline structure at position C-6 after biosynthesis of the polyketide backbone is completed. A recombinant strain of S. Read More

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November 2005

Induction of artificial endometrial cycles with oestradiol implants and injectable progesterone: establishment of a viable pregnancy in a woman with 17-alpha-hydroxylase deficiency.

Hum Reprod 1995 Sep;10(9):2456-8

Department of Obstetrics and Gynecology, Sapir Medical Center, Kfar Saba, Israel.

Repeated attempts with oral oestrogens and injectable progesterone failed to induce secretory endometrium in a woman with 17-alpha-hydroxylase deficiency. The insertion of s.c. Read More

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September 1995

Disorders of steroid 17 alpha-hydroxylase deficiency.

Endocrinol Metab Clin North Am 1994 Jun;23(2):341-57

Department of Medicine, Escola Paulista de Medicina, São Paulo, Brazil.

The human P450c17 alpha gene (CYP17) is a single copy gene located in chromosome 10, consisting of 8 exons and 7 introns. 17 alpha-Hydroxylase/17,20-lyase deficiency is one of two hypertensive forms of congenital adrenal hyperplasia and is inherited as an autosomal recessive trait; although rare, it probably exists with twice the frequency of the 11 beta-hydroxylase deficiency. Deficient 17 alpha-hydroxylation of pregnenolone and progesterone and subsequent deficiency of the cleavage of the C-17,20 carbon bond result in the absence of sex hormone formation in both the adrenal glands and the gonads, causing hypogonadism and male pseudohermaphroditism. Read More

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Pituitary-ovarian responses to nafarelin testing in the polycystic ovary syndrome.

N Engl J Med 1989 Mar;320(9):559-65

Department of Obstetrics/Gynecology, University of Chicago Pritzker School of Medicine, IL.

To investigate the basis of polycystic ovary syndrome, we examined the responses of patients to nafarelin, a specific gonadotropin-releasing-hormone agonist, given to stimulate pituitary and gonadal secretion. We compared 16 normal women in the follicular phase, 5 normal men, 8 women with polycystic ovary syndrome, and 1 woman with polycystic ovary syndrome caused by a 3 beta-hydroxysteroid dehydrogenase deficiency. After 100 micrograms of nafarelin was given subcutaneously, serum follicle-stimulating hormone and luteinizing hormone increased rapidly to peak levels within four hours. Read More

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Effects of tocopherol depletion on the regional differences in adrenal microsomal lipid peroxidation and steroid metabolism.

Endocrinology 1988 Aug;123(2):975-80

Department of Biomedical Sciences, University of Illinois College of Medicine, Rockford 61107.

Studies were done to assess the contribution of alpha-tocopherol to the regional differences in microsomal lipid peroxidation (LP) and steroid metabolism in the guinea pig adrenal cortex. In normal guinea pigs, ferrous ion (Fe2+)- and ascorbic acid-induced LP are far greater in microsomal preparations from the inner adrenal zone (zona reticularis) than in those from the outer zones (zona fasciculata plus zona glomerulosa). The amounts of unsaturated fatty acids, substrates for LP, are similar in the two zones, but alpha-tocopherol concentrations are 4-5 times greater in outer than inner zone microsomes. Read More

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Reduced activity of androgen biosynthesis in the testes of rats with analbuminemia.

J Steroid Biochem 1986 Apr;24(4):871-6

Steroid metabolism in Nagase Analbuminemia Rats (NAR), a mutant strain established from Sprague-Dawley rats, was studied. NAR are characterized by lack of serum albumin and hyperlipidemia. Total testosterone concentration in the serum of NAR was lower than that of normal rats, while the serum free testosterone, LH and FSH concentrations were similar. Read More

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Determination of steroid profiles in healthy and diseased states: identification and quantitation of a block of 17 alpha-hydroxylase.

Clin Chim Acta 1983 Mar;129(1):3-11

The steroid metabolic profile on a patient with a suspected block in steroid biosynthesis was analyzed by gas chromatography and gas chromatography-mass spectrometry. The results of this work led to an interpretation of a block at the 17 alpha-hydroxylase step. Although the steroid metabolic profile was complex, we could not detect any steroids with a hydroxy moiety at position C-17. Read More

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