Results 161 to 170 of about 4,036 (198)
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Steroid 21 Hydroxylase Deficiency Congenital Adrenal Hyperplasia
Endocrinology and Metabolism Clinics of North America, 2009Steroid 21 hydroxylase deficiency is the most common form of congenital adrenal hyperplasia (CAH). The severity of this disorder depends on the extent of impaired enzymatic activity, which is caused by various mutations of the 21 hydroxylase gene. This article reviews adrenal steroidogenesis and the pathophysiology of 21 hydroxylase deficiency.
Saroj, Nimkarn +2 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 ...
M I, New, P W, Speiser
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Factors influencing steroid hydroxylases in liver microsomes
Advances in Enzyme Regulation, 1966Abstract Liver microsomal enzymes oxidatively metabolize drugs, insecticides and steroids in the presence of TPNH and oxygen to compounds that are more polar than the substrate. Several factors listed below which alter the activity of the oxidative drug-metabolizing enzymes in liver microsomes, similarly alter the activity of liver microsomal enzymes
R, Kuntzman, R, Welch, A H, Conney
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Steroid 16α‐Hydroxylase from Human Fetal Liver; Inhibition by Steroids
Acta Obstetricia et Gynecologica Scandinavica, 1980The 16α‐hydroxylase system in fetal liver which used dehydroepiandrosterone (DHA) or pregnenolone as substrate, was apparently inhibited by various endogenous and synthetic steroids: DHA, pregnenolone, their sulfates, androstenediol, androstenetriol, estrone, estradiol‐17β, ethynylestradiol and chlormadinone‐acetate.
Y, Sano +5 more
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Urinary steroid excretion in 17α-hydroxylase deficiency
Journal of Steroid Biochemistry, 1978Abstract Urinary steroids from a patient with 17α-hydroxylase deficiency syndrome have been identified. The steroids were obtained by enzymatic hydrolysis of urine, Amberlite XAD-2 extraction and Sephadex LH-20 column chromatography. Following preparation of volatile derivatives, the steroids were separated and estimated by gas chromatography on open-
J W, Honour +3 more
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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 ...
J, Neunzig, R, Bernhardt
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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 21-hydroxylase deficiency (congenital adrenal hyperplasia)
The American Journal of Medicine, 1995Congenital adrenal hyperplasia, caused by any one of a number of inborn errors of steroidogenesis in which cortisol is not sufficiently produced by the adrenal cortex, is in most cases due to a deficiency of the enzyme steroid 21-hydroxylase. Classic 21-hydroxylase deficiency occurs in about 1 in 14,000 live births. In classic 21-hydroxylase deficiency,
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Genetics of steroid 21-hydroxylase deficiency
Trends in Genetics, 1985Abstract Classical and non-classical adrenal hyperplasia due to steroid 21-hydroxylase deficiency (210HD) are HLA-linked, autosomal recessive disorders characterized by a variable degree of hyperandrogenism and cortisol deficiency. Direct screening of newborns has placed the incidence of the classical disease at 1 : 5000 to 1 : 15 000, whereas the ...
Phyllis W. Speiser, Maria I. New
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Human saturated steroid 6alpha-hydroxylase.
The Journal of clinical endocrinology and metabolism, 1997This study was conducted to evaluate further the reaction catalyzed by the saturated steroid 6alpha-hydroxylase of extrahepatic human tissues. Progesterone and 5alpha-dihydroprogesterone (5alpha-DHP) are plasma-borne precursors of 5alpha-pregnan-3alpha-ol-20-one, an anxiolytic/anesthetic steroid, and 5alpha-pregnan-3beta-ol-20-one in extrahepatic human
R, Dombroski, M L, Casey, P C, Macdonald
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