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Regulation of 21-Hydroxylase activity by steroids
Endocrine Research, 1995In this study, we investigated the effect of steroids on guinea pig and bovine adrenal steroidogenesis, especially 21-hydroxylase activity. Analysis of guinea pig adrenal steroids indicated the presence of high concentrations of androstenedione in the guinea pig adrenal; furthermore, in vitro studies using guinea pig adrenal cortex cells in primary ...
A, Bélanger +4 more
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Mutations in Steroid 21-Hydroxylase (CYP21)
Human Mutation, 1994The inherited inability to synthesize cortisol is termed congenital adrenal hyperplasia. More than 90% of cases are caused by 21-hydroxylase deficiency. This syndrome is characterized by signs of androgen excess and often mineralocorticoid deficiency. Steroid 21-hydroxylase (P450c21) is a microsomal enzyme expressed in the adrenal gland that catalyzes ...
P C, White +3 more
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2017
Steroid hormones play an important role in the survival of the individual and of species. This is realized by the regulation of salt and sugar homeostasis by mineralocorticosteroids and glucocorticosteroids and of reproductive processes by sex hormones.
Hofland, J., de Jong, F. H.
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Steroid hormones play an important role in the survival of the individual and of species. This is realized by the regulation of salt and sugar homeostasis by mineralocorticosteroids and glucocorticosteroids and of reproductive processes by sex hormones.
Hofland, J., de Jong, F. H.
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Steroid 21-Hydroxylase Deficiency in Mice*
Endocrinology, 1988The enzyme steroid 21-hydroxylase (21-OHase) plays a key role in adrenal steroidogenesis. Defects in this enzyme are responsible for one of the most common inborn errors of metabolism in humans. Duplicated genes for the enzyme are located in the class III region of the major histocompatibility complex (MHC), HLA.
H, Gotoh +4 more
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Molecular Cloning of Steroid 21-Hydroxylase
Endocrine Research, 1984Congenital adrenal hyperplasia due to 21-hydroxylase (21-OH) deficiency is HLA-linked. The haplotype HLA-(A3);Bw47;DR7 is strongly associated with 21-OH deficiency and always carries a null allele at the complement C4A (Rodgers) locus. It seemed likely that this haplotype carries a deletion encompassing both the C4A and 21-OH loci. We hypothesized that
P C, White, B, Dupont, M I, New
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Disorders of steroid 11β-hydroxylase isozymes
Trends in Endocrinology & Metabolism, 1992Steroid 11 beta-hydroxylase activity in the adrenal cortex is required for the synthesis of the major glucocorticoids and mineralocorticoids, but different isozymes mediate this conversion in the zona fasciculata, where cortisol is produced, and the zona glomerulosa, the site of aldosterone synthesis.
P C, White, L, Pascoe
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Disorders of Steroid 11β-Hydroxylase Isozymes*
Endocrine Reviews, 1994The most active corticosteroids are 11 beta-hydroxylated. Humans have two isozymes with 11 beta-hydroxylase activity that are respectively required for cortisol and aldosterone synthesis. CYP11B1 (11 beta-hydroxylase) is expressed at high levels and is regulated by ACTH, whereas CYP11B2 (aldosterone synthase) is normally expressed at low levels and is ...
P C, White, K M, Curnow, L, Pascoe
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An Inhibitor of Adrenal Steroid 11β-Hydroxylase
Nature, 1964Trans-1,4-bis (2-chlorobenzylaminomethyl) cyclohexane dihydrochloride (‘AY–9944’)1 has been shown to represent a novel class of cholesterol biosynthesis inhibitors which act by interfering with the enzymatic conversion of 7-dehydrocholesterol to cholesterol2,3. In laboratory animals, ‘AY–9944’ significantly depressed serum sterol-levels4.
M L, GIVNER +3 more
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Steroid 11β-hydroxylase deficiency and related disorders
Endocrinology and Metabolism Clinics of North America, 2001Three disorders result from mutations involving two closely linked 11 beta-hydroxylase genes. Steroid 11 beta-hydroxylase deficiency results from mutations in CYP11B1. This is a form of congenital adrenal hyperplasia (CAH) characterized by hypertension and signs of androgen excess. Mutations in CYP11B2 cause aldosterone synthase deficiency, an isolated
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