• 3-Beta-hydroxysteroid dehydrogenase (3BHSD) deficiency is a rare form of congenital adrenal hyperplasia that results in decreased production of all three groups of adrenal steroids: mineralocorticoids, glucocorticoids, and sex steroids. (medscape.com)
  • Although first described in male infants with ambiguous genitalia and severe salt wasting, 3-beta-hydroxysteroid dehydrogenase deficiency also occurs in 46,XX female infants (who may have mild clitoromegaly), as well as in older patients who present with a milder or so-called late-onset variant. (medscape.com)
  • A variety of mutations in the HSD3B2 gene affect the activity of this enzyme, resulting in the extremely variable, phenotypic presentations of 3-beta-hydroxysteroid dehydrogenase deficiency. (medscape.com)
  • Adrenal androgens undergo peripheral conversion to testosterone and dihydrotestosterone, and steroid precursors produce specific findings depending upon the exact enzyme deficiency. (health.am)
  • Disease severity depends on the specific CYP21A2 mutation and degree of enzyme deficiency. (merckmanuals.com)
  • there is complete deficiency of enzyme activity that leads to very low levels of cortisol and aldosterone. (merckmanuals.com)
  • Micropenis can also occur in children with LH-receptor defects and defects in testosterone biosynthesis (e.g. 17-beta hydroxysteroid dehydrogenase deficiency). (medscape.com)
  • Individuals with 17-beta hydroxysteroid dehydrogenase deficiency most often have female-appearing genitalia and, less often, ambiguous genitalia. (medscape.com)
  • Deficiency of any of these enzymes or transport proteins leads to partial or complete loss of their activity. (iitta.gov.ua)
  • In AGS, there are distinguished a phenotype and nonclassical forms of steroidogenesis enzyme deficiency. (iitta.gov.ua)
  • 17-β-Hydroxysteroid dehydrogenase type 3 (17βHSD-3) is present almost exclusively in the testes, and converts androstenedione (A) to testosterone (T). 17βHSD-3 deficiency is rare. (degruyter.com)
  • 4. Rosler A. 17 beta-hydroxysteroid dehydrogenase3 deficiency in the Mediterranean population. (degruyter.com)
  • Phenotypic variability in 17beta-hydroxysteroid dehydrogenase-3 deficiency and diagnostic pitfalls. (degruyter.com)
  • Familial male pseudohermaphroditism with gynaecomastia due to 17 betahydroxysteroid dehydrogenase deficiency. (degruyter.com)
  • 17betahydroxysteroid dehydrogenase 3 deficiency in a male pseudohermaphrodite. (degruyter.com)
  • 17p-Hydroxysteroid dehydrogenase type 3 deficiency as a result of a homozygous 7 base pair deletion in 17 β HSD3 gene. (degruyter.com)
  • 11. George MM, New MI, Ten S, Sultan C, Bhangoo A. The clinical and molecular heterogeneity of 17βHSD-3 enzyme deficiency. (degruyter.com)
  • 12. Bertelloni S, Dati E, Hiort O. Diagnosis of 17 beta-hydroxysteroid dehydrogenase deficiency. (degruyter.com)
  • Molecular genetics and pathophysiology of 17 beta-hydroxysteroid dehydrogenase 3 deficiency. (degruyter.com)
  • Elevated pregnenolone levels occur in different forms of congenital adrenal hyperplasia, due to 3β-hydroxysteroid dehydrogenase deficiency or 17α-hydroxylase deficiencies. (athenslab.gr)
  • This condition is due to defects in type II 3-beta-hydroxysteroid dehydrogenase, an enzyme that occurs almost exclusively in the gonads and adrenal glands. (medscape.com)
  • Older patients with mild defects in 3-beta-hydroxysteroid dehydrogenase activity (late-onset or nonclassic variant) may present with premature pubic hair development, hirsutism, irregular menstrual cycles or primary amenorrhea. (medscape.com)
  • GA inhibits 11 beta-hydroxysteroid dehydrogenase, resulting in inhibition of the conversion of cortisol to the inactive steroid cortisone and elevated cortisol levels. (psychosocialsomatic.com)
  • In addition, GA inhibits 17,20-lyase and 17 beta-hydroxysteroid dehydrogenase, resulting in decreased conversions of 17-hydroxyprogesterone to androstenedione and androstenedione to testosterone. (psychosocialsomatic.com)
  • Comment: Microsomal enzyme 11-beta-hydroxysteroid dehydrogenase type 1 (11-beta-HSD-1) required for cortisone conversion to its active metabolite, cortisol, in hepatic and adipose tissue. (sideload.com)
  • 11β-hydroxysteroid dehydrogenase/reductase (11β-HSD) enzymes 1 and 2 regulate the amount of cortisone and cortisol in human tissues. (helsinki.fi)
  • Testosterone is peripherally converted by the enzyme 5-alpha reductase to the more potent androgen DHT, which is responsible for virilization of the male external genitalia. (medscape.com)
  • Expression levels of mRNA for neurosteroidogenic enzymes 17β-HSD, 5α-reductase, 3α-HSD and cytochrome P450 aromatase in the fetal wild type and SF-1 knockout mouse brain. (arnes.si)
  • The neuroactive progestogen allopregnanolone (also known as 3,5-tetrahydroprogesterone or 3-hydroxy-5-pregnan-20-one) is synthesized from progesterone by isozymes on the enzyme 5alpha-reductase (5-reductase) and by the enzyme 3alpha-hydroxysteroid dehydrogenase (3-HSD). (deubiquitinaseinhibitor.com)
  • The enzyme kinetics for warfarin reduction were evaluated in both fractions, and formation of warfarin alcohols was used as an indicator of hepatic reductase activity. (aspetjournals.org)
  • Steroid sex hormones are detected in corals and sea anemones even though these animals do not have estrogen receptors and their repertoire of steroidogenic enzymes appears to be incomplete. (sciencegate.app)
  • Results show transcription of steroidogenic enzymes, and cholesterol binding/transport proteins have similar transcription profiles for E2, T, and cholesterol treatments, but different profiles when BP-3 or BBP is present. (sciencegate.app)
  • Gene and protein expression of steroidogenic enzymes were determined. (sciencegate.app)
  • Steroidogenic enzymes were expressed in human AT with depot-specific differences. (sciencegate.app)
  • Expression of steroidogenic enzymes, including steroidogenic acute regulatory protein, cholesterol side chain cleavage enzyme, and 3β-hydroxysteroid dehydrogenase type 1 messenger RNA, were examined by real-time polymerase chain reaction after 6 h of human chorionic gonadotropin treatment. (utoledo.edu)
  • The ovaries were resected, pooled according to prolactin levels and microsomal enzyme activities were measured from each pool. (elsevierpure.com)
  • Other names in common use include 20alpha-hydroxy steroid dehydrogenase, 20alpha-hydroxy steroid dehydrogenase, 20alpha-HSD, and 20alpha-HSDH. (wikipedia.org)
  • This enzyme participates in c21-steroid hormone metabolism. (wikipedia.org)
  • Predicted to enable oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor and steroid dehydrogenase activity. (nih.gov)
  • A steroid novice experimenting with this agent is likely to gain 20 to 30 pounds of massive bulk, and it can often be accomplished within 6 weeks of use. (decatest.net)
  • 28-Homobrassinolide boosted the activity of 3beta-hydroxysteroid dehydrogenase and 17beta-hydroxy steroid dehydrogenase in the rats' testes. (ironmagazine.com)
  • Abi blocks 17α-hydroxylase/17,20-lyase (CYP17A1), an enzyme required for androgen synthesis. (aacc.org)
  • Piperine in vitro also increased androgen production and stimulated cholesterol side-chain cleavage enzyme and 17α-hydroxylase/20-lyase activities in immature Leydig cells. (frontiersin.org)
  • In the simple virilizing form, cortisol synthesis is impaired, leading to increased androgen activity, but there is sufficient enzyme activity to maintain normal, or only slightly decreased, aldosterone production. (merckmanuals.com)
  • For example, CYP2E1 is the gene that encodes the enzyme CYP2E1 - one of the enzymes involved in paracetamol (acetaminophen) metabolism. (wikidoc.org)
  • Only 60% of yeast and 73% of the human RBPs have functions assigned to RNA biology or structural motifs known to convey RNA binding, and many intensively studied proteins surprisingly emerge as RBPs (termed 'enigmRBPs'), including almost all glycolytic enzymes, pointing to emerging connections between gene regulation and metabolism. (nature.com)
  • atazanavir will increase the level or effect of vamorolone by affecting hepatic/intestinal enzyme CYP3A4 metabolism. (medscape.com)
  • chloramphenicol will increase the level or effect of vamorolone by affecting hepatic/intestinal enzyme CYP3A4 metabolism. (medscape.com)
  • clarithromycin will increase the level or effect of vamorolone by affecting hepatic/intestinal enzyme CYP3A4 metabolism. (medscape.com)
  • 20alpha-HSD has been initially described as a progesterone metabolizing enzyme of the ovary. (wikipedia.org)
  • Progesterone production was analyzed by an enzyme immunoassay kit after human chorionic gonadotropin (50 ng/ml) or forskolin (10 μM) treatment for 24 h. (utoledo.edu)
  • Pregnenolone may either be converted to 17-OH pregnenolone via the enzymatic action of 17α-hydroxylase or to progesterone via the enzymatic action of 3β-hydroxysteroid dehydrogenase. (athenslab.gr)
  • This enzyme system mediates the conversion of pregnenolone to progesterone in the adrenal gland. (vin.com)
  • By contrast, cortisol synthesis and secretion is regulated by adrenocorticotropic hormone (ACTH), which stimulates the enzyme P-450scc (20,22 desmolase), with subsequent increased production of all adrenal steroids in both the zona fasciculata and the zona reticularis (see image below). (medscape.com)
  • The enzymatic defects causing female virilization involve 3β-hydroxysteroid dehydrogenase Δ5-Δ4 isomerase (3β-HSD), P 450 C21 hydroxylase (21-OH), and P 450 C11 hydroxylase (11-OH). (health.am)
  • The enzyme cytochrome P450 RORγ Modulator Species aromatase (AROM) can then synthesize estrogens fromAlcohol. (deubiquitinaseinhibitor.com)
  • To investigate whether hyperprolactinemia directly affects rat ovarian function, we examined the ovarian histopathology and the activities of the four ovarian enzymes 3β-hydroxysteroid dehydrogenase (3β-HSD), 17-hydroxylase (17-OH), 17,20-desmolase (17,20-D) and aromatase in hyperprolactinemic rats and controls. (elsevierpure.com)
  • ACTH stimulates the enzyme P-450scc (20,22 desmolase) with subsequent increased production of all adrenal steroids. (medscape.com)
  • 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) is a key enzyme that transform cortisone to cortisol, which activates the endogenous glucocorticoid function. (ijbs.com)
  • There is an additional effect more distal in the enzyme cascade and possibly also on the interconversion of cortisol to cortisone. (vin.com)
  • 11βHSD1 is an ER-bound enzyme catalyzing the conversion of inactive cortisone in active cortisol in humans. (biomedcentral.com)
  • The primary antioxidant found in licorice root, the flavonoid glabridin, may inactivate or inhibit the activities of some cytochrome P450 enzymes. (psychosocialsomatic.com)
  • The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. (origene.com)
  • 8. Rosler A, Silverstein S, Abeliovich D. A (R80Q) mutation in 17 betahydroxysteroid dehydrogenase type 3 gene among Arabs of Israel is associated with pseudohermaphroditism in males and normal asymptomatic females. (degruyter.com)
  • Genes encoding CYP enzymes, and the enzymes themselves, are designated with the abbreviation "CYP", followed by an Arabic numeral indicating the gene family, a capital letter indicating the subfamily, and another numeral for the individual gene. (wikidoc.org)
  • However, some gene or enzyme names for CYPs may differ from this nomenclature, denoting the catalytic activity and the name of the compound used as substrate. (wikidoc.org)
  • The researchers also discovered that the brassinosteroid boosted the activity of endogenous antioxidant enzymes like superoxide dismutase, catalase and GSH, and reduced the peroxidation of fats, particularly in the testes of the rats with diabetes. (ironmagazine.com)
  • The GOIs are 17β-hydroxysteroid dehydrogenases type 14 (17β HSD14) and type 12 (17β HSD12), Niemann-Pick C type 2 (NPC2), Equistatin (EI), Complement component C3 (C3), Cathepsin L (CTSL), Patched domain-containing protein 3 (PTCH3), Smoothened (SMO), Desert Hedgehog (DHH), Zinc finger protein GLI2 (GLI2), and Vitellogenin (VTG). (sciencegate.app)
  • Treatment with human chorionic gonadotropin increased the expression of downstream targets of mammalian target of rapamycin complex 1, as well as cholesterol side chain cleavage enzyme, 3β-hydroxysteroid dehydrogenase type 1 and steroidogenic acute regulatory protein messenger RNAs. (utoledo.edu)
  • STX2171, a 17β-hydroxysteroid dehydrogenase type 3 inhibitor, is efficacious in vivo in a novel hormone-dependent prostate cancer model. (ox.ac.uk)
  • G6P availability directly modulates the activity of 11β-hydroxysteroid dehydrogenase type 1 (11βHSD1), an ER-bound enzyme playing a key role in the development of the metabolic syndrome (MS). (biomedcentral.com)
  • Consistent with increase in serum T, piperine increased Leydig cell number, cell size, and multiple steroidogenic pathway proteins, including steroidogenic acute regulatory protein, cholesterol side-chain cleavage enzyme, 3β-hydroxysteroid dehydrogenase 1, 17α-hydroxylase/20-lyase, and steroidogenic factor 1 expression levels. (frontiersin.org)
  • Expressions of phospho-ribosomal protein S6 kinase and cholesterol side chain cleavage enzyme were analyzed after 15 min and 24 h of human chorionic gonadotropin treatment, respectively. (utoledo.edu)
  • d ) Validation of the yeast mRNA interactome using western blotting of input samples and eluate after interactome capture with specific antibodies (ADH1, alcohol dehydrogenase 1, PUB1) or against TAP-tagged proteins (PGK1, phosphoglycerate kinase 1, TDH1, triose phosphate dehydrogenase, TRX2, thioredoxine 2, SHE2, Swi5p-dependent HO Expression 2). (nature.com)
  • Chronic kidney disease (CKD) affects the nonrenal clearance of drugs by modulating the functional expression of hepatic drug-metabolizing enzymes and transporters. (aspetjournals.org)
  • Moreover, our recent transcriptomic analysis reveals a decline in mRNA levels of Cyp17a1 , encoding a steroidogenic enzyme, in 4- to 30-day cultures of 6 d pp mouse testes ( 10 ). (elifesciences.org)
  • Cells were cultured for 72 h and treated with human chorionic gonadotropin (50 ng/ml) for different time periods with or without pretreatment with mammalian target of rapamycin complex 1 inhibitor, rapamycin, (20 nM) for 1 h. (utoledo.edu)
  • From studies in humans and various other species it is known that trilostane is a competitive inhibitor of 3β-hydroxysteroid-dehydrogenase. (vin.com)
  • The difference between 11β-HSD1 and 2 ligand binding sites is unknown, which complicates the search of selective inhibitors to both of the enzymes. (helsinki.fi)
  • More recent incretin-based treatment strategies include glucagon-like peptide-1 (GLP-1) mimetics and inhibitors of the enzyme that degrades GLP-1, dipeptidyl peptidase-4 (DPP-4). (ddw-online.com)
  • Male pseudohermaphroditism caused by mutations of testicular 17-β-hydroxysteroid dehydrogenase 3. (degruyter.com)
  • 1, 2, 3] Approximately 20% of the genes expressed in human leukocytes are regulated positively or negatively by glucocorticoids. (medscape.com)
  • In enzymology, a 20-α-hydroxysteroid dehydrogenase (EC 1.1.1.149) is an enzyme that catalyzes the chemical reaction 17alpha,20alpha-dihydroxypregn-4-en-3-one + NAD(P)+ ⇌ {\displaystyle \rightleftharpoons } 17alpha-hydroxyprogesterone + NAD(P)H + H+ The 3 substrates of this enzyme are 17alpha,20alpha-dihydroxypregn-4-en-3-one, NAD+, and NADP+, whereas its 4 products are 17-alpha-hydroxyprogesterone, NADH, NADPH, and H+. (wikipedia.org)
  • The enzyme 17β-hydroxysteroid dehydrogenase (17β-HSD) catalyzes the reversible interconversion of E1 and E2. (scirp.org)
  • An NAD + dependent enzyme that catalyzes the oxidation of 3-carboxy-2-hydroxy-4-methylpentanoate to 3-carboxy-4-methyl-2-oxopentanoate. (nih.gov)
  • This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with NAD+ or NADP+ as acceptor. (wikipedia.org)
  • Increased ACTH secretion also produces overproduction of both the adrenal steroids preceding the missing enzyme and those not requiring the missing enzyme (ie, build-up of compounds both before the block and "sideways" from the block). (medscape.com)
  • Increased ACTH secretion results in overproduction of adrenal steroids preceding the missing enzyme as well as those not requiring the missing enzyme. (medscape.com)
  • Enzymes stimulated by adrenocorticotropic hormone (ACTH). (merckmanuals.com)
  • We show that this conserved RNA interactome harbours many proteins without previously assigned roles in RNA biology (enigmRBPs), including surprisingly many metabolic enzymes. (nature.com)
  • 17β-Hydroxysteroid dehydrogenases (17β-HSDs) catalyse the 17-position reduction/oxidation of steroids. (ox.ac.uk)
  • Its production occurs in the mitochondria by cleavage of the C-20 side chain of cholesterol by the P-450SCC enzyme. (athenslab.gr)
  • In the XX newborn, gonads are not palpable, which classically leads to the diagnosis of female pseudohermaphroditism. (health.am)
  • Human placental 17 beta-estradiol dehydrogenase and 20 alpha-hydroxysteroid dehydrogenase. (wikipedia.org)
  • Dihydrotestosterone and mestanolone are both very weak in this regard, owing to the fact that these molecules are not very stable in the high enzyme (3-alpha hydroxysteroid dehydrogenase) environment of muscle tissue. (decatest.net)
  • The difference between the enzymes could not be identified by visual inspections Therefore, seven compounds, of which six are 11β-HSD2 -selective, were docked both to 11β-HSD1 and 11β-HSD2 ligand binding sites using the program GOLD. (helsinki.fi)
  • The docking results revealed that the compounds orientate differently in the enzymes. (helsinki.fi)
  • Recent studies in humans and rodents suggest a role of 11β-hydroxysteroid dehydrogenase (11β-HSD) in the development of idiopathic obesity and MS [ 2 ]. (biomedcentral.com)
  • Ensure the patients' samples, calibrators, and controls are at ambient temperature (20-25°C) before measurement. (cdc.gov)
  • In addition, the enzyme reduces and inactivates 17-deoxycorticosterone, the precursor of aldosterone and corticosterone. (wikipedia.org)
  • [ 4 ] in which activity of one of the enzymes necessary for cortisol production is deficient (see image below). (medscape.com)
  • 11βHSD1 requires NADPH as a cofactor generated by the hexose-6-phosphate dehydrogenase (H6PDH)-mediated conversion of glucose 6-phosphate (G6P) to 6-phosphogluconactone (6PGL) [ 10 ]. (biomedcentral.com)