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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-02-12 21:58:27 UTC
HMDB IDHMDB0000319
Secondary Accession Numbers
  • HMDB00319
Metabolite Identification
Common Name18-Hydroxycorticosterone
Description18-Hydroxycorticosterone is a corticosteroid and a derivative of corticosterone. If it is present in sufficiently high concentrations, it can lead to serious electrolyte imbalances (an electrolyte toxin). 18-Hydroxycorticosterone serves as an intermediate in the synthesis of aldosterone by the enzyme aldosterone synthase in the zona glomerulosa. Chronically high levels of 18-hydroxycorticosterone are associated with at least three inborn errors of metabolism including adrenal hyperplasia type V, corticosterone methyl oxidase I deficiency, and corticosterone methyl oxidase II deficiency. Each of these conditions is characterized by excessive amounts of sodium being released in the urine (salt wasting), along with insufficient release of potassium in the urine, usually beginning in the first few weeks of life. This imbalance leads to low levels of sodium and high levels of potassium in the blood (hyponatremia and hyperkalemia, respectively). Individuals with corticosterone methyloxidase deficiency can also have high levels of acid in the blood (metabolic acidosis). Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). The hyponatremia, hyperkalemia, and metabolic acidosis associated with corticosterone methyloxidase deficiency can cause nausea, vomiting, dehydration, low blood pressure, extreme tiredness (fatigue), and muscle weakness.
Structure
Thumb
Synonyms
ValueSource
11b,18,21-Trihydroxy-pregn-4-ene-3,20-dioneHMDB
18 HydrocorticosteroneMeSH
18 HydroxycorticosteroneMeSH
18-HydrocorticosteroneMeSH
Hydroxycorticosterone, 18MeSH
(11β)-11,18,21-Trihydroxypregn-4-ene-3,20-dioneCAS
(11beta)-11,18,21-Trihydroxypregn-4-ene-3,20-dioneCAS
Chemical FormulaC21H30O5
Average Molecular Weight362.4599
Monoisotopic Molecular Weight362.20932407
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number561-65-9
SMILES
[H][C@@]12CC[C@H](C(=O)CO)[C@@]1(CO)C[C@H](O)[C@@]1([H])[C@@]2([H])CCC2=CC(=O)CC[C@]12C
InChI Identifier
InChI=1S/C21H30O5/c1-20-7-6-13(24)8-12(20)2-3-14-15-4-5-16(18(26)10-22)21(15,11-23)9-17(25)19(14)20/h8,14-17,19,22-23,25H,2-7,9-11H2,1H3/t14-,15-,16+,17-,19+,20-,21+/m0/s1
InChI KeyHFSXHZZDNDGLQN-ZVIOFETBSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as 21-hydroxysteroids. These are steroids carrying a hydroxyl group at the 21-position of the steroid backbone.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSteroids and steroid derivatives
Sub ClassHydroxysteroids
Direct Parent21-hydroxysteroids
Alternative Parents
Substituents
  • Progestogin-skeleton
  • 21-hydroxysteroid
  • Pregnane-skeleton
  • 20-oxosteroid
  • 18-hydroxysteroid
  • 3-oxo-delta-4-steroid
  • 3-oxosteroid
  • 11-hydroxysteroid
  • 11-beta-hydroxysteroid
  • Oxosteroid
  • Delta-4-steroid
  • Cyclohexenone
  • Alpha-hydroxy ketone
  • Cyclic alcohol
  • Ketone
  • Secondary alcohol
  • Cyclic ketone
  • Hydrocarbon derivative
  • Organic oxide
  • Carbonyl group
  • Organic oxygen compound
  • Alcohol
  • Organooxygen compound
  • Primary alcohol
  • Aliphatic homopolycyclic compound
Molecular FrameworkAliphatic homopolycyclic compounds
External Descriptors
Ontology
Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

Physiological effect

Health effect:

  Health condition:

    Endocrine disorders:

Disposition

Biological location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

    Endocrine gland:

  Tissue and substructures:

  Cell and elements:

Source:

Role

Industrial application:

Biological role:

  Molecular messenger:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.15 g/LALOGPS
logP1.28ALOGPS
logS-3.4ALOGPS
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-1639000000-7012f18a6579e07dc571View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positivesplash10-03di-1410290000-5789e767e5a0058f10edView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01r2-0019000000-8a12570d1c1a01069d09View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-004j-0119000000-2b8864299061070cced5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0019-3494000000-ebcb1ff70c533c4c9459View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-0009000000-0a027947a1d058fb84b7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-03dl-1019000000-cb3605cb617647c036e0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-05fr-5094000000-8d05b88622baf6731c7fView in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Adrenal Gland
Pathways
NameSMPDB/PathwhizKEGG
11-beta-hydroxylase deficiency (CYP11B1)Pw000551Pw000551 greyscalePw000551 simpleNot Available
17-alpha-hydroxylase deficiency (CYP17)Pw000542Pw000542 greyscalePw000542 simpleNot Available
21-hydroxylase deficiency (CYP21)Pw000552Pw000552 greyscalePw000552 simpleNot Available
3-Beta-Hydroxysteroid Dehydrogenase DeficiencyPw000695Pw000695 greyscalePw000695 simpleNot Available
Adrenal Hyperplasia Type 3 or Congenital Adrenal Hyperplasia due to 21-hydroxylase DeficiencyPw000177Pw000177 greyscalePw000177 simpleNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.000550-0.00138 uMChildren (1 - 13 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.030 (0.012-0.057) uMChildren (1-13 years old)BothTerminal aldosterone biosynthesis defects details
Associated Disorders and Diseases
Disease References
Terminal aldosterone biosynthesis defects
  1. Peter M, Partsch CJ, Sippell WG: Multisteroid analysis in children with terminal aldosterone biosynthesis defects. J Clin Endocrinol Metab. 1995 May;80(5):1622-7. [PubMed:7745009 ]
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB021945
KNApSAcK IDNot Available
Chemspider ID10748
KEGG Compound IDC01124
BioCyc ID18-HYDROXYCORTICOSTERONE
BiGG IDNot Available
Wikipedia Link18-Hydroxycorticosterone
METLIN ID5308
PubChem Compound11222
PDB IDNot Available
ChEBI ID16485
References
Synthesis ReferenceBoudi, Ahmed; Lemoine, Pascale; Viossat, Bernard; Tomas, Alain; Fiet, Jean; Galons, Herve. A convenient synthesis of 18-hydroxycorticosterone and 18-hydroxy-11-desoxycorticosterone via stereospecific hypoiodination of 20-hydroxysteroids. Tetrahedron (1999), 55(16), 5171-5176.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Sonino N, Chow D, Levine LS, New MI: Clinical response to metyrapone as indicated by measurement of mineralocorticoids and glucocorticoids in normal children. Clin Endocrinol (Oxf). 1981 Jan;14(1):31-9. [PubMed:7226574 ]
  2. Vecsei P, Abdelhamid S, Mittelstadt GV, Lichtwald K, Haack D, Lewicka S: Aldosterone metabolites and possible aldosterone precursors in hypertension. J Steroid Biochem. 1983 Jul;19(1A):345-51. [PubMed:6887870 ]
  3. Kooner JS, Few JD, Lee CY, Taylor GM, James VH: Investigation of the salivary 18-hydroxycorticosterone:aldosterone ratio in man using a direct assay. J Steroid Biochem Mol Biol. 1991 Mar;38(3):377-82. [PubMed:2009228 ]
  4. Gomez-Sanchez CE, Clore JN, Estep HL, Watlington CO: Effect of chronic adrenocorticotropin stimulation on the excretion of 18-hydroxycortisol and 18-oxocortisol. J Clin Endocrinol Metab. 1988 Aug;67(2):322-6. [PubMed:2839536 ]
  5. Honda M, Tsuchiya M, Tamura H, Watanabe H, Izumi Y, Hatano M, Shiratsuchi T, Den K, Kawaoi A, Okano T: In vivo and in vitro studies on steroid metabolism in a case of primary aldosteronism with multiple lesions of adenoma and nodular hyperplasia. Endocrinol Jpn. 1982 Oct;29(5):529-40. [PubMed:6303762 ]
  6. Vecsei P, Benraad TJ, Hofman J, Abdelhamid S, Haack D, Lichtwald K: Direct radioimmunoassays for "aldosterone" and "18-hydroxycorticosterone" in unprocessed urine, and their use in screening to distinguish primary aldosteronism from hypertension. Clin Chem. 1982 Mar;28(3):453-6. [PubMed:7067086 ]

Enzymes

General function:
Involved in monooxygenase activity
Specific function:
Has steroid 11-beta-hydroxylase activity. In addition to this activity, the 18 or 19-hydroxylation of steroids and the aromatization of androstendione to estrone have also been ascribed to cytochrome P450 XIB.
Gene Name:
CYP11B1
Uniprot ID:
P15538
Molecular weight:
57572.44
General function:
Involved in monooxygenase activity
Specific function:
Preferentially catalyzes the conversion of 11-deoxycorticosterone to aldosterone via corticosterone and 18-hydroxycorticosterone.
Gene Name:
CYP11B2
Uniprot ID:
P19099
Molecular weight:
57559.62
Reactions
Corticosterone + reduced adrenal ferredoxin + Oxygen → 18-Hydroxycorticosterone + oxidized adrenal ferredoxin + Waterdetails
Corticosterone + Reduced adrenal ferredoxin + Oxygen → 18-Hydroxycorticosterone + Oxidized adrenal ferredoxin + Waterdetails
18-Hydroxycorticosterone + Reduced adrenal ferredoxin + Oxygen → Aldosterone + Oxidized adrenal ferredoxin + Waterdetails