Results 151 to 160 of about 4,036 (198)

[29] Adrenal steroid hydroxylases

1969
Publisher Summary This chapter discusses the general procedures used in the laboratories for separating mitochondria and microsomes from the adrenal cortex of steers and for preparing a reaction medium for the hydroxylase assays. The chapter summarizes the most common steroid assays. Modifications of these procedures are pointed out on the individual
Otto Rosenthal, Shakunthala Narasimhulu
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Disorders of steroid 11β-hydroxylase isozymes

Trends in Endocrinology & Metabolism, 1992
Steroid 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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Steroid 21-Hydroxylase

1993
Cortisol synthesis in the zona fasciculata of the adrenal cortex requires five enzymatic steps: the cholesterol side chain is cleaved to yield pregnenolone, which is dehydrogenated at the 3β position to progesterone, which is successively hydroxylated at the 17α, 21 and 11β positions. The side-chain cleavage and the three hydroxylations are mediated by
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Steroid 11β-hydroxylase deficiency and related disorders

Endocrinology and Metabolism Clinics of North America, 2001
Three 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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Bovine steroid 21-hydroxylase: regulation of biosynthesis

Biochemistry, 1986
A recombinant cDNA clone, PBC21-1, specific for bovine steroid 21-hydroxylase cytochrome P-450 (P-450C21) was identified in a bovine adrenocortical cDNA library, and this identity was confirmed by nucleotide sequencing which revealed significant amino acid homology (77%) with human P-450C21 cDNA.
M E, John, T, Okamura, A, Dee, B, Adler, M C, John, P C, White, E R, Simpson, M R, Waterman   +7 more
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Steroid 11β- hydroxylase deficiency congenital adrenal hyperplasia

Trends in Endocrinology & Metabolism, 2008
Congenital adrenal hyperplasia due to steroid 11beta-hydroxylase deficiency is a genetic disorder of steroidogenesis, transmitted as an autosomal recessive trait. It is associated with low renin hypertension, hypokalemia, hyperandrogenemia and genital ambiguity in affected females. Mutations in the CYP11B1 gene, causing 11beta-hydroxylase deficiency in
Saroj, Nimkarn, Maria I, New
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Regulation of 21-Hydroxylase activity by steroids

Endocrine Research, 1995
In 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, Y, Tremblay, M, Vallée, P H, Provencher, S, Perron   +4 more
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Steroid 11β-Hydroxylase Deficiency

2001
Inherited defects in cortisol biosynthesis are collectively termed congenital adrenal hyperplasia [reviewed in (1)]. Steroid 21-hydroxylase deficiency is, by far, the most common of these defects; it is reviewed elsewhere in this volume. This chapter reviews a related disease, steroid 11β-hydroxylase deficiency [reviewed in greater detail in (2)].
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Molecular pathology of steroid 21-hydroxylase deficiency

The Journal of Steroid Biochemistry and Molecular Biology, 1991
The molecular pathology of steroid 21-hydroxylase deficiency is attributable to unequal crossover-mediated gene deletion or to large- or small-scale replacement of the functional CYP21B gene sequence by a copy of the analogous CYP21A pseudogene sequence.
T, Strachan, P C, White
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Genetics of Steroid 21-Hydroxylase Deficiency

1987
Publisher Summary Congenital adrenal hyperplasia because of steroid 21-hydroxylase (21-OHase) deficiency is one of the most common inborn errors of metabolism. The 21-OHase locus is closely linked to the HLA major histocompatibility complex, and 21-OHase deficiency alleles show characteristic associations with particular HLA antigens and with alleles
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