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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:30:37 UTC
HMDB IDHMDB01170
Secondary Accession NumbersNone
Metabolite Identification
Common NameLathosterol
DescriptionLathosterol 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 ). Plasma lathosterol levels are significantly elevated in patients with bile acid malabsorption (PMID: 8777839 ). 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
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 FormulaC27H46O
Average Molecular Weight386.6535
Monoisotopic Molecular Weight386.354866094
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 Namelathosterol
CAS Registry Number80-99-9
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 KeyIZVFFXVYBHFIHY-SKCNUYALSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassSteroids and Steroid Derivatives
Sub ClassCholesterols and Derivatives
Other Descriptors
  • Aliphatic Homopolycyclic Compounds
Substituents
  • 3 Hydroxy Steroid
  • Bicyclohexane
  • Cyclic Alcohol
  • Cyclohexane
  • Cyclohexene
  • Secondary Alcohol
  • Sesterterpene
Direct ParentCholesterols and Derivatives
Ontology
StatusDetected 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
StateSolid
Experimental Properties
PropertyValueReference
Melting Point122.0 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility3.500E-05 g/LALOGPS
logP7.4ALOGPS
logP7.11ChemAxon
logS-7ALOGPS
pKa (Strongest Acidic)18.36ChemAxon
pKa (Strongest Basic)-1.3ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count1ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area20.23ChemAxon
Rotatable Bond Count5ChemAxon
Refractivity120.62ChemAxon
Polarizability50.6ChemAxon
Spectra
Spectra1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane (predicted from logP)
  • Endoplasmic reticulum
Biofluid Locations
  • Blood
Tissue Location
  • Adipose Tissue
  • Adrenal Cortex
  • Adrenal Gland
  • Adrenal Medulla
  • Bladder
  • Brain
  • Eye Lens
  • Fetus
  • Fibroblasts
  • Gonads
  • Gut
  • Intestine
  • Kidney
  • Liver
  • Muscle
  • Myelin
  • Nerve Cells
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Skin
Pathways
NameSMPDB LinkKEGG Link
Steroid BiosynthesisSMP00023map00100
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified9.9 +/- 7.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified5.38 +/- 0.038 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified2.43 +/- 0.048 uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified67.5 +/- 58.4 uMAdult (>18 years old)Both
Cerebrotendinous xanthomatosis (CTX)
details
BloodDetected and Quantified9.6 (5.4-13.5) uMAdult (>18 years old)Female
Cirrhosis
details
Associated Disorders and Diseases
Disease References
Cerebrotendinous xanthomatosis
  1. 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
Cirrhosis
  1. 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
Associated OMIM IDs
  • 213700 (Cerebrotendinous xanthomatosis)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022463
KNApSAcK IDNot Available
Chemspider ID59151
KEGG Compound IDC01189
BioCyc IDCPD-4186
BiGG ID37036
Wikipedia LinkNot Available
NuGOwiki LinkHMDB01170
Metagene LinkHMDB01170
METLIN ID6052
PubChem Compound65728
PDB IDNot Available
ChEBI ID17168
References
Synthesis ReferenceFieser, 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. 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
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  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
  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
  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

Enzymes

General function:
Involved in iron ion binding
Specific function:
Catalyzes a dehydrogenation to introduce C5-6 double bond into lathosterol.
Gene Name:
SC5DL
Uniprot ID:
O75845
Molecular weight:
35300.55
Reactions
Lathosterol + NAD(P)H + Oxygen → 7-Dehydrocholesterol + NAD(P)(+) + Waterdetails
Lathosterol + NADH + Hydrogen Ion + Oxygen → 7-Dehydrocholesterol + NAD + Waterdetails
Lathosterol + NADPH + Hydrogen Ion + Oxygen → 7-Dehydrocholesterol + NADP + Waterdetails
General function:
Involved in iron ion binding
Specific function:
Not Available
Gene Name:
MSMO1
Uniprot ID:
Q15800
Molecular weight:
19470.325
General function:
Involved in 3-beta-hydroxy-delta5-steroid dehydrogenase activity
Specific function:
Not Available
Gene Name:
NSDHL
Uniprot ID:
Q15738
Molecular weight:
41899.99
General function:
Involved in oxidoreductase activity
Specific function:
Responsible for the reduction of the keto group on the C-3 of sterols.
Gene Name:
HSD17B7
Uniprot ID:
P56937
Molecular weight:
38205.77
General function:
Involved in cholestenol delta-isomerase activity
Specific function:
Catalyzes the conversion of Delta(8)-sterols to their corresponding Delta(7)-isomers.
Gene Name:
EBP
Uniprot ID:
Q15125
Molecular weight:
26352.615
Reactions
Lathosterol → 5-alpha-cholest-8-en-3-beta-oldetails
Lathosterol → 5a-Cholest-8-en-3b-oldetails
General function:
Energy production and conversion
Specific function:
Catalyzes the reduction of the delta-24 double bond of sterol intermediates. Protects cells from oxidative stress by reducing caspase 3 activity during apoptosis induced by oxidative stress. Also protects against amyloid-beta peptide-induced apoptosis.
Gene Name:
DHCR24
Uniprot ID:
Q15392
Molecular weight:
60100.805
Reactions
Lathosterol + NADP → 5-alpha-Cholesta-7,24-dien-3-beta-ol + NADPHdetails
Lathosterol + NADP → 5a-Cholesta-7,24-dien-3b-ol + NADPH + Hydrogen Iondetails