Page last updated: 2024-10-06

3-beta-Hydroxysteroid Dehydrogenase Deficiency

Proteins (11)

ProteinSynonymsTaxonomy
Steroid 21-hydroxylaseEC 1.14.14.16; 21-OHase; Cytochrome P-450c21; Cytochrome P450 21; Cytochrome P450 XXI; Cytochrome P450-C21; Cytochrome P450-C21BHomo sapiens (human)
3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 13 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type I; 3-beta-HSD I; 3-beta-hydroxy-5-ene steroid dehydrogenase; 3-beta-hydroxy-Delta(5)-steroid dehydrogenase; 1.1.1.145; 3-beta-hydroxysteroid 3-dehydrogenase; 1.1.1.270; Delta-5-3-ketosteroid iHomo sapiens (human)
Aldo-keto reductase family 1 member C4EC 1.1.1.-; EC 1.1.1.209; EC 1.1.1.210; EC 1.1.1.51; EC 1.1.1.53; EC 1.1.1.62; 3-alpha-hydroxysteroid dehydrogenase type I; 3-alpha-HSD1; 1.1.1.357; 3alpha-hydroxysteroid 3-dehydrogenase; Chlordecone reductase; CDR; 1.1.1.225; Dihydrodiol dehydrogenase 4;Homo sapiens (human)
Corticosteroid 11-beta-dehydrogenase isozyme 1EC 1.1.1.146; 11-beta-hydroxysteroid dehydrogenase 1; 11-DH; 11-beta-HSD1; Short chain dehydrogenase/reductase family 26C member 1Homo sapiens (human)
Cytochrome P450 11B1, mitochondrialCYPXIB1; Cytochrome P-450c11; Cytochrome P450C11; Steroid 11-beta-hydroxylase, CYP11B1; 1.14.15.4Homo sapiens (human)
Cytochrome P450 11B2, mitochondrialAldosterone synthase; ALDOS; Aldosterone-synthesizing enzyme; CYPXIB2; Corticosterone 18-monooxygenase, CYP11B2; 1.14.15.5; Cytochrome P-450Aldo; Cytochrome P-450C18; Steroid 11-beta-hydroxylase, CYP11B2; 1.14.15.4; Steroid 18-hydroxylaseHomo sapiens (human)
Corticosteroid 11-beta-dehydrogenase isozyme 2EC 1.1.1.-; 11-beta-hydroxysteroid dehydrogenase type 2; 11-DH2; 11-beta-HSD2; 11-beta-hydroxysteroid dehydrogenase type II; 11-HSD type II; 11-beta-HSD type II; NAD-dependent 11-beta-hydroxysteroid dehydrogenase; 11-beta-HSD; Short chain dehydrogenase/reHomo sapiens (human)
Steroid 17-alpha-hydroxylase/17,20 lyaseEC 1.14.14.19; 17-alpha-hydroxyprogesterone aldolase; 1.14.14.32; CYPXVII; Cytochrome P450 17A1; Cytochrome P450-C17; Cytochrome P450c17; Steroid 17-alpha-monooxygenaseHomo sapiens (human)
3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 23 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type II; 3-beta-HSD II; 3-beta-HSD adrenal and gonadal typeHomo sapiens (human)
Cholesterol side-chain cleavage enzyme, mitochondrialEC 1.14.15.6; CYPXIA1; Cholesterol desmolase; Cytochrome P450 11A1; Cytochrome P450(scc)Homo sapiens (human)
Aldo-keto reductase family 1 member D1EC 1.3.1.3; 3-oxo-5-beta-steroid 4-dehydrogenase; Delta(4)-3-ketosteroid 5-beta-reductase; Delta(4)-3-oxosteroid 5-beta-reductaseHomo sapiens (human)

Compounds (24)

CompoundDescription
hydronium ion
cortisoneA naturally occurring glucocorticoid that has been used in replacement therapy for ADRENAL INSUFFICIENCY and as an anti-inflammatory agent. Cortisone itself is inactive; it is converted in the liver to the active metabolite HYDROCORTISONE. (From Martindale, The Extra Pharmacopoeia, 30th ed, p726)
NADH
HydrocortisoneThe main glucocorticoid secreted by the ADRENAL CORTEX. Its synthetic counterpart is used, either as an injection or topically, in the treatment of inflammation, allergy, collagen diseases, asthma, adrenocortical deficiency, shock, and some neoplastic conditions.
ProgesteroneThe major progestational steroid that is secreted primarily by the CORPUS LUTEUM and the PLACENTA. Progesterone acts on the UTERUS, the MAMMARY GLANDS and the BRAIN. It is required in EMBRYO IMPLANTATION; PREGNANCY maintenance, and the development of mammary tissue for MILK production. Progesterone, converted from PREGNENOLONE, also serves as an intermediate in the biosynthesis of GONADAL STEROID HORMONES and adrenal CORTICOSTEROIDS.
CholesterolThe principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.
desoxycorticosteroneA steroid metabolite that is the 11-deoxy derivative of CORTICOSTERONE and the 21-hydroxy derivative of PROGESTERONE
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
21-deoxycortisol, (11alpha)-isomer
18-hydroxycorticosterone11 beta,18,21-Trihydroxypregn-4-ene-3,20-dione.
isocaproaldehydeproduced from side-chain cleavage of 20,22-dihydroxycholesterol
21-hydroxypregnenoloneRN given refers to (3beta)-isomer;
cortodoxone17,21-Dihydroxypregn-4-ene-3,20-dione. A 17-hydroxycorticosteroid with glucocorticoid and anti-inflammatory activities.
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
17-alpha-hydroxyprogesteroneA metabolite of PROGESTERONE with a hydroxyl group at the 17-alpha position. It serves as an intermediate in the biosynthesis of HYDROCORTISONE and GONADAL STEROID HORMONES.
5-dihydrocortisoneRN for cpd without isomeric designation not in Chemline 9/85
5 beta-dihydrocortisol
(22r)-22-hydroxycholesterol
17, 21-dihydroxypregnenolone
corticosteroneAn adrenocortical steroid that has modest but significant activities as a mineralocorticoid and a glucocorticoid. (From Goodman and Gilman's The Pharmacological Basis of Therapeutics, 8th ed, p1437)
creolinfrom refined coal tar oils
17-alpha-hydroxypregnenoloneA 21-carbon steroid that is converted from PREGNENOLONE by STEROID 17-ALPHA-HYDROXYLASE. It is an intermediate in the delta-5 pathway of biosynthesis of GONADAL STEROID HORMONES and the adrenal CORTICOSTEROIDS.
5-alpha-dihydroprogesteroneA biologically active 5-alpha-reduced metabolite of plasma PROGESTERONE. It is the immediate precursor of 5-alpha-pregnan-3-alpha-ol-20-one (ALLOPREGNANOLONE), a neuroactive steroid that binds with GABA(A) RECEPTOR.