Results 151 to 160 of about 509,948 (192)
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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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Endocrine Research, 1991
Over long periods of growth in culture, bovine adrenocortical cells lose the ability to express the steroid hydroxylase genes. For 17 alpha-hydroxylase, cells show a stochastic pattern of phenotypic switching from a state in which they express this gene ...
P. Hornsby
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Over long periods of growth in culture, bovine adrenocortical cells lose the ability to express the steroid hydroxylase genes. For 17 alpha-hydroxylase, cells show a stochastic pattern of phenotypic switching from a state in which they express this gene ...
P. Hornsby
semanticscholar +1 more source
Steroid 17α-hydroxylase of the rat adrenal
Journal of Steroid Biochemistry, 1979Abstract The 105,000 g microsomal fraction of male or female rat adrenal contains steroid 17α-hydroxylase activity, as measured by a tritium exchange assay using 17α-[ 3 H]-pregnenolone as substrate. Administration of estrogen caused a pronounced increase in enzyme activity in the adrenals of both sexes, but the increase was greater in males. Ovine
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A new ELISA for autoantibodies to steroid 21-hydroxylase
Clinical Chemistry and Laboratory Medicine (CCLM), 2017Abstract Background: A new ELISA for autoantibodies to steroid 21-hydroxylase (21-OH Ab) is described. Methods: In the assay test sample autoantibodies form a bridge between 21-OH coated onto the plate well and liquid phase 21-OH ...
Maria Del Pilar Larosa+11 more
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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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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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Molecular pathology of steroid 21-hydroxylase deficiency
The Journal of Steroid Biochemistry and Molecular Biology, 1991The 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.
Tom Strachan, Perrin C. White
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Bovine steroid 21-hydroxylase: regulation of biosynthesis
Biochemistry, 1986A 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.
Maliyakal E. John+8 more
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Genetics of Adrenal Steroid 21-Hydroxylase Deficiency*
Endocrine Reviews, 1986Impairment of 21-hydroxylation is the most common enzymatic deficiency resulting in the syndrome of CAH, which may present either in the classical form in infants or in the nonclassical form in older individuals. Variable signs and symptoms of androgen excess are common to both types of the disorder, which are transmitted as autosomal recessive traits ...
Phyllis W. Speiser, Maria I. New
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Genetics of Steroid 21-Hydroxylase Deficiency
1987Publisher 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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Effect of sulfonated steroids on steroidogenic cytochrome P450-dependent steroid hydroxylases
The Journal of Steroid Biochemistry and Molecular Biology, 2018In the last decades, sulfonated steroids evolved from inactive metabolites intended for excretion to highly relevant compounds involved in many physiological processes. Investigations of the impact of sulfonated steroids on the steroid hormone biosynthesis revealed that, on the one hand, these can serve as substrate for steroidogenic cytochromes P450 ...
Rita Bernhardt, Jens Neunzig
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