Human Metabolome Database Version 3.5

Showing metabocard for Lathosterol (HMDB01170)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:30:37 -0600
HMDB ID HMDB01170
Secondary Accession Numbers None
Metabolite Identification
Common Name Lathosterol
Description Lathosterol is a a sterol (a combination steroid and alcohol) and a lipid found in the cell membranes of all body tissues, and transported in the blood plasma of all animals. It is used as an indicator of whole-body cholesterol synthesis (PMID 14511438 Link_out). Plasma lathosterol levels are significantly elevated in patients with bile acid malabsorption (PMID: 8777839 Link_out). Lathosterol oxidase (EC 1.14.21.6) is an enzyme that catalyzes the chemical reaction 5alpha-cholest-7-en-3beta-ol + NAD(P)H + H+ + O2 cholesta-5,7-dien-3beta-ol + NAD(P)+ + 2 H2O.
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (3beta)-Cholest-7-en-3-ol
  2. (3beta,5alpha)-Cholest-7-en-3-ol
  3. (3beta,alpha)-Cholest-7-en-3-ol
  4. (7)-Cholestenol
  5. 3b-Hydroxy-5-cholestene
  6. 3beta-Hydroxy-5alpha-cholest-7-ene
  7. 5-a-Cholest-7-en-3-beta-ol
  8. 5-alpha-Cholest-7-en-3-beta-ol
  9. 5alpha-cholest-7-en-3beta-ol
  10. 7-Cholesten-3-beta-ol
  11. 7-Cholestenol
  12. 7-Cholesterol
  13. Cholest-7-en-3-ol
  14. Cholest-7-en-3beta-ol
  15. Cholest-7-en-ol
  16. Cholesterol - synthetic
  17. Cholesterol extra pure
  18. Cholesterol gr
  19. Cholesterol Pharma
  20. Cholesterol,NF Grade
  21. delta(7)-Cholestenol
  22. DELTA7-Cholesten-3beta-ol
  23. DELTA7-Cholestenol
  24. gamma-Cholestenol
  25. gamma-Cholesterol
  26. Lathosterol
Chemical Formula C27H46O
Average Molecular Weight 386.6535
Monoisotopic Molecular Weight 386.354866094
IUPAC Name (1R,2S,5S,7S,11R,14R,15R)-2,15-dimethyl-14-[(2R)-6-methylheptan-2-yl]tetracyclo[8.7.0.0^{2,7}.0^{11,15}]heptadec-9-en-5-ol
Traditional IUPAC Name lathosterol
CAS Registry Number 80-99-9
SMILES [H][C@@]1(CC[C@@]2([H])C3=CC[C@@]4([H])C[C@@H](O)CC[C@]4(C)[C@@]3([H])CC[C@]12C)[C@H](C)CCCC(C)C
InChI Identifier InChI=1S/C27H46O/c1-18(2)7-6-8-19(3)23-11-12-24-22-10-9-20-17-21(28)13-15-26(20,4)25(22)14-16-27(23,24)5/h10,18-21,23-25,28H,6-9,11-17H2,1-5H3/t19-,20+,21+,23-,24+,25+,26+,27-/m1/s1
InChI Key IZVFFXVYBHFIHY-SKCNUYALSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Lipids
Class Steroids and Steroid Derivatives
Sub Class Cholesterols and Derivatives
Other Descriptors
  • Aliphatic Homopolycyclic Compounds
Substituents
  • 3 Hydroxy Steroid
  • Bicyclohexane
  • Cyclic Alcohol
  • Cyclohexane
  • Cyclohexene
  • Secondary Alcohol
  • Sesterterpene
Direct Parent Cholesterols and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Hormones, Membrane component
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane (predicted from logP)
  • Endoplasmic reticulum
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 122.0 °C Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 3.500E-05 g/L ALOGPS
LogP 7.40 ALOGPS
LogP 7.11 ChemAxon
LogS -7.04 ALOGPS
pKa (strongest acidic) 18.36 ChemAxon
pKa (strongest basic) -1.3 ChemAxon
Hydrogen Acceptor Count 1 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 20.23 A2 ChemAxon
Rotatable Bond Count 5 ChemAxon
Refractivity 120.62 ChemAxon
Polarizability 50.6 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
1H NMR Spectrum
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane (predicted from logP)
  • Endoplasmic reticulum
Biofluid Locations
  • Blood
Tissue Location
  • Muscle
  • Skeletal Muscle
  • Bladder
  • Fibroblasts
  • Intestine
  • Neuron
  • Pancreas
  • Placenta
  • Kidney
  • Liver
  • Brain
  • Myelin
  • Prostate
  • Adrenal Gland
  • Fetus
  • Skin
  • Adipose Tissue
  • Adrenal Cortex
  • Adrenal Medulla
  • Nerve Cells
  • Platelet
  • Gonads
  • Gut
  • Eye Lens
Pathways
Name SMPDB Link KEGG Link
Steroid Biosynthesis SMP00023 map00100 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
9.9 +/- 7.0 uM Adult (>18 years old) Both Comment Normal
Blood Detected and Quantified
5.38 +/- 0.038 uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
2.43 +/- 0.048 uM Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 67.5 +/- 58.4 uM Adult (>18 years old) Both Comment Cerebrotendinous xanthomatosis (CTX)
Blood Detected and Quantified 9.6 (5.4-13.5) uM Adult (>18 years old) Female Comment Cirrhosis
Associated Disorders and Diseases
Disease References
Cerebrotendinous xanthomatosis
  • Halperin G, Elisaf M, Leitersdorf E, Harats D: A new method for determination of serum cholestanol by high-performance liquid chromatography with ultraviolet detection. J Chromatogr B Biomed Sci Appl. 2000 Jun 9;742(2):345-52. Pubmed: 10901139 Link_out
      Cirrhosis
      • Halperin G, Elisaf M, Leitersdorf E, Harats D: A new method for determination of serum cholestanol by high-performance liquid chromatography with ultraviolet detection. J Chromatogr B Biomed Sci Appl. 2000 Jun 9;742(2):345-52. Pubmed: 10901139 Link_out
          Associated OMIM IDs
          • 213700 Link_out (Cerebrotendinous xanthomatosis)
          DrugBank ID Not Available
          DrugBank Metabolite ID Not Available
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB022463
          KNApSAcK ID Not Available
          Chemspider ID 59151 Link_out
          KEGG Compound ID C01189 Link_out
          BioCyc ID CPD-4186 Link_out
          BiGG ID 37036 Link_out
          Wikipedia Link Not Available
          NuGOwiki Link HMDB01170 Link_out
          Metagene Link HMDB01170 Link_out
          METLIN ID 6052 Link_out
          PubChem Compound 65728 Link_out
          PDB ID Not Available
          ChEBI ID 17168 Link_out
          References
          Synthesis Reference Fieser, Louis F. Cholesterol and companions. III. Cholestanol, lathosterol, and ketone 104. Journal of the American Chemical Society (1953), 75 4395-403.
          Material Safety Data Sheet (MSDS) Download (PDF)
          General References
          1. Paiva H, Thelen KM, Van Coster R, Smet J, De Paepe B, Mattila KM, Laakso J, Lehtimaki T, von Bergmann K, Lutjohann D, Laaksonen R: High-dose statins and skeletal muscle metabolism in humans: a randomized, controlled trial. Clin Pharmacol Ther. 2005 Jul;78(1):60-8. Pubmed: 16003294 Link_out
          2. Rioux FM, Innis SM: Cholesterol and fatty acid metabolism in piglets fed sow milk or infant formula with or without addition of cholesterol. Metabolism. 1993 Dec;42(12):1552-9. Pubmed: 8246769 Link_out
          3. Laaksonen R, Thelen KM, Paiva H, Matinheikki J, Vesalainen R, Janatuinen T, Knuuti J, Rontu R, von Bergmann K, Lutjohann D, Lehtimaki T: Genetic variant of the SREBF-1 gene is significantly related to cholesterol synthesis in man. Atherosclerosis. 2006 Mar;185(1):206-9. Epub 2005 Jul 11. Pubmed: 16005884 Link_out
          4. Nikkila K, Hockerstedt K, Miettinen TA: Liver transplantation modifies serum cholestanol, cholesterol precursor and plant sterol levels. Clin Chim Acta. 1992 Jun 30;208(3):205-18. Pubmed: 1499139 Link_out
          5. Lutjohann D, Harzer K, Gieselmann V, Eckhardt M: Reduced brain cholesterol content in arylsulfatase A-deficient mice. Biochem Biophys Res Commun. 2006 Jun 2;344(2):647-50. Epub 2006 Apr 7. Pubmed: 16630546 Link_out
          6. Brunetti-Pierri N, Corso G, Rossi M, Ferrari P, Balli F, Rivasi F, Annunziata I, Ballabio A, Russo AD, Andria G, Parenti G: Lathosterolosis, a novel multiple-malformation/mental retardation syndrome due to deficiency of 3beta-hydroxysteroid-delta5-desaturase. Am J Hum Genet. 2002 Oct;71(4):952-8. Epub 2002 Aug 20. Pubmed: 12189593 Link_out
          7. Hamilton JJ, Synnes A, Innis SM: Plasma cholesterol and lathosterol levels in term infants in the early neonatal period. Pediatr Res. 1992 Apr;31(4 Pt 1):396-400. Pubmed: 1570206 Link_out
          8. Boomsma DI, Princen HM, Frants RR, Gevers Leuven JA, Kempen HJ: Genetic analysis of indicators of cholesterol synthesis and absorption: lathosterol and phytosterols in Dutch twins and their parents. Twin Res. 2003 Aug;6(4):307-14. Pubmed: 14511438 Link_out
          9. Farkkila MA, Kairemo KJ, Taavitsainen MJ, Strandberg TA, Miettinen TA: Plasma lathosterol as a screening test for bile acid malabsorption due to ileal resection: correlation with 75SeHCAT test and faecal bile acid excretion. Clin Sci (Lond). 1996 Apr;90(4):315-9. Pubmed: 8777839 Link_out

          Enzymes
          Name: Lathosterol oxidase
          Reactions:
          Lathosterol + NAD(P)H + Oxygen unknown 7-Dehydrocholesterol + NAD(P)(+) + Water details
          Lathosterol + NADH + Hydrogen Ion + Oxygen unknown 7-Dehydrocholesterol + NAD + Water details
          Lathosterol + NADPH + Hydrogen Ion + Oxygen unknown 7-Dehydrocholesterol + NADP + Water details
          Gene Name: SC5DL
          Uniprot ID: O75845 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Methylsterol monooxygenase 1
          Reactions: Not Available
          Gene Name: MSMO1
          Uniprot ID: Q15800 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating
          Reactions: Not Available
          Gene Name: NSDHL
          Uniprot ID: Q15738 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: 3-keto-steroid reductase
          Reactions: Not Available
          Gene Name: HSD17B7
          Uniprot ID: P56937 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase
          Reactions:
          Lathosterol unknown 5-alpha-cholest-8-en-3-beta-ol details
          Lathosterol unknown 5a-Cholest-8-en-3b-ol details
          Gene Name: EBP
          Uniprot ID: Q15125 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Delta(24)-sterol reductase
          Reactions:
          Lathosterol + NADP unknown 5-alpha-Cholesta-7,24-dien-3-beta-ol + NADPH details
          Lathosterol + NADP unknown 5a-Cholesta-7,24-dien-3b-ol + NADPH + Hydrogen Ion details
          Gene Name: DHCR24
          Uniprot ID: Q15392 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA