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Human Metabolome Database Version 2.5

 

Showing metabocard for 24-Hydroxycholesterol (HMDB01419)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2006-08-16 15:44:11
Update Date 2011-01-26 11:01:42
Accession Number HMDB01419
Secondary Accession Numbers Not Available
Common Name 24-Hydroxycholesterol
Description 24-Hydroxycholesterol (24OHC) is almost exclusively formed in the brain. The enzymatic conversion of CNS cholesterol to 24OHC, which readily crosses the blood-brain barrier, is the major pathway for brain cholesterol elimination and brain cholesterol homeostasis maintenance. The enzyme mediating this conversion has been characterized at the molecular level as cholesterol 24-hydroxylase (EC 1.14.13.98, CYP46) and is mainly located in neurons. Like other oxysterols, 24OHC is efficiently converted into normal bile acids or excreted in bile in its sulfated and glucuronidated form. Levels of 24OHC in the circulation decrease with age in infants and children. In adults, however, the levels appear to be stable. There is accumulating evidence pointing toward a potentially important link between cholesterol, beta-amyloid, and Alzheimer's disease. Patients with active demyelinating diseases had increased levels of 24OHC in cerebrospinal fluid (CSF). Patients with Alzheimer's disease have slightly increased levels of 24OHC in CSF. Patients with multiple sclerosis have a tendency to have higher levels of 24OHC during active periods. (PMID: 15061359, 14574622)
Synonyms
  1. (24S)-24-Hydroxycholesterol
  2. 24(S)-Hydroxycholesterol
  3. 24-Hydroxycholesterol
  4. 24S-Cholest-5-ene-3b,24-diol
  5. Cerebrosterin
  6. Cerebrosterol
  7. Cholest-5-ene-3b,24b-diol
  8. 24S-hydroxycholesterol
  9. (24S)-cholest-5-ene-3b,24-diol
  10. (24S)-cholest-5-ene-3beta,24-diol
  11. Cholest-5-ene-3,24-diol
  12. Cholest-5-en-3b,24S-diol
  13. Cholest-5-en-3beta,24S-diol
Chemical IUPAC Name (3S,8S,9S,10R,13R,14S,17R)-17-[(2R,5S)-5-hydroxy-6-methyl-heptan-2-yl]-10,13-dimethyl-2,3,4,7,8,9,11,12,14,15,16,17-dodecahydro-1H-cyclopenta[a]phenanthren-3-ol
Chemical Formula C27H46O2
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Cholesterols and derivatives
Class
  • Steroids and Steroid Derivatives
Sub Class
  • Dihydroxy steroids
Family
  • Mammalian Metabolite
Species
  • secondary alcohol
  • alkene
Biofunction
  • Hormones, Membrane component
Application
—
Source
  • Endogenous
Average Molecular Weight 402.653
Monoisotopic Molecular Weight 402.349792
Isomeric SMILES CC(C)[C@@H](O)CC[C@@H](C)[C@H]1CC[C@H]2[C@@H]3CC=C4C[C@@H](O)CC[C@]4(C)[C@H]3CC[C@]12C
Canonical SMILES CC(C)C(O)CCC(C)C1CCC2C3CC=C4CC(O)CCC4(C)C3CCC12C
KEGG Compound ID C13550 Link Image
BioCyc ID CPD-7239 Link Image
BiGG ID 2197434 Link Image
Wikipedia Link Not Available
NuGOwiki Link HMDB01419 Link Image
Metagene Link HMDB01419 Link Image
METLIN ID Not Available
PubChem Compound 121948 Link Image
PubChem Substance 7846926 Link Image
ChEBI ID 34310 Link Image
CAS Registry Number 474-73-7
InChI Identifier InChI=1/C27H46O2/c1-17(2)25(29)11-6-18(3)22-9-10-23-21-8-7-19-16-20(28)12-14-26(19,4)24(21)13-15-27(22,23)5/h7,17-18,20-25,28-29H,6,8-16H2,1-5H3/t18-,20+,21+,22-,23+,24+,25+,26+,27-/m1/s1
Synthesis Reference Prasad V V; Ponticorvo L; Lieberman S Identification of 24-hydroxycholesterol in bovine adrenals in both free and esterified forms and in bovine brains as its sulfate ester. Journal of steroid biochemistry (1984), 21(6), 733-6.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 4.03e-04 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge 0
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity 6.5 [Predicted by PubChem via XLOGP]; 5.70 [Predicted by ALOGPS] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show Link Image
SDF File Show Link Image
PDB File Show Link Image
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • endoplasmic reticulum
Biofluid Location
  • Blood
  • Cerebrospinal Fluid
Tissue Location Not Available
Concentrations (Normal)
Biofluid Blood
Value 0.0018 +/- 0.00052 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Holdenrieder S, Lutjohann D, Geiger S, von Bergmann K, Stieber P, Hamann GF: Does brain specific 24S-hydroxycholesterol in plasma indicate the disruption of the blood-brain barrier in patients with ischemic stroke? Neurosci Lett. 2004 Sep 23;368(2):201-4. [PubMed Link Image]
Biofluid CSF
Value 0.0034 (0.0033-0.0036) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Concentrations (Abnormal)
Biofluid Blood
Value 0.11 +/- 0.03 uM
Age Adult:>18 yrs old
Sex N/A
Condition Hypercholesterolemia
Comments Moderately elevated plasma cholesterol levels
References
  • Thelen KM, Laaksonen R, Paiva H, Lehtimaki T, Lutjohann D: High-dose statin treatment does not alter plasma marker for brain cholesterol metabolism in patients with moderately elevated plasma cholesterol levels. J Clin Pharmacol. 2006 Jul;46(7):812-6. [PubMed Link Image]
Biofluid CSF
Value 0.0061 (0.0058-0.0064) uM
Age Elderly:>65 yrs old
Sex Both
Condition Alzheimer's disease
Comments Not Available
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 0.016 (0.014-0.017) uM
Age Adult:>18 yrs old
Sex Both
Condition Demyelinating polyneuropathy
Comments Not Available
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 0.0045 (0.0041-0.0050) uM
Age Adult:>18 yrs old
Sex Both
Condition Subarachnoid hemorrhage
Comments Not Available
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 0.0086 (0.0076-0.0096) uM
Age Adult:>18 yrs old
Sex Both
Condition Meningitis
Comments Viral
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 0.0062 (0.0057-0.0068) uM
Age Adult:>18 yrs old
Sex Both
Condition Neuroborreliosis
Comments Not Available
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 0.0077 (0.0066-0.0093) uM
Age Adult:>18 yrs old
Sex N/A
Condition Mild cognitive impairment
Comments Also known as incipient dementia or isolated memory impairment
References
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 0.004 +/- 0.0013 uM
Age Adult:>18 yrs old
Sex Both
Condition Multiple sclerosis
Comments Not Available
References
  • Leoni V, Lutjohann D, Masterman T: Levels of 7-oxocholesterol in cerebrospinal fluid are more than one thousand times lower than reported in multiple sclerosis. J Lipid Res. 2005 Feb;46(2):191-5. Epub 2004 Dec 1. [PubMed Link Image]
Associated Disorders
Condition References
Alzheimer's disease
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Demyelinating polyneuropathy
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Hypercholesterolemia
  • Thelen KM, Laaksonen R, Paiva H, Lehtimaki T, Lutjohann D: High-dose statin treatment does not alter plasma marker for brain cholesterol metabolism in patients with moderately elevated plasma cholesterol levels. J Clin Pharmacol. 2006 Jul;46(7):812-6. [PubMed Link Image]
Meningitis
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Mild cognitive impairment
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Multiple sclerosis
  • Leoni V, Lutjohann D, Masterman T: Levels of 7-oxocholesterol in cerebrospinal fluid are more than one thousand times lower than reported in multiple sclerosis. J Lipid Res. 2005 Feb;46(2):191-5. Epub 2004 Dec 1. [PubMed Link Image]
Neuroborreliosis
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Subarachnoid hemorrhage
  • Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
OMIM ID
Pathways
Name SMPDB Link KEGG Link
Bile Acid Biosynthesis SMP00035 Link Image map00120 Link Image
General References
  1. Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed Link Image]
Metabolic Enzymes
  1. Not Available
  2. Cholesterol 24-hydroxylase
Enzyme 1 [top]
Enzyme 1 ID 8685
Enzyme 1 Name Not Available
Enzyme 1 Synonyms Not Available
Enzyme 1 Gene Name Not Available
Enzyme 1 Protein Sequence Not Available
Enzyme 1 Number of Residues Not Available
Enzyme 1 Molecular Weight Not Available
Enzyme 1 Theoretical pI Not Available
Enzyme 1 GO Classification Not Available
Enzyme 1 General Function Not Available
Enzyme 1 Specific Function Not Available
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function Not Available
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein Not Available
Enzyme 1 UniProtKB/Swiss-Prot ID Not Available
Enzyme 1 UniProtKB/Swiss-Prot Entry Name Not Available
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence Not Available
Enzyme 1 GenBank Gene ID Not Available
Enzyme 1 GeneCard ID Not Available
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Not Available
Enzyme 1 Locus Not Available
Enzyme 1 SNPs Not Available
Enzyme 1 General References Not Available
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 10660
Enzyme 2 Name Cholesterol 24-hydroxylase
Enzyme 2 Synonyms
  1. CH24H
  2. Cytochrome P450 46A1
Enzyme 2 Gene Name CYP46A1
Enzyme 2 Protein Sequence >Cholesterol 24-hydroxylase 
MSPGLLLLGSAVLLAFGLCCTFVHRARSRYEHIPGPPRPSFLLGHLPCFWKKDEVGGRVL
QDVFLDWAKKYGPVVRVNVFHKTSVIVTSPESVKKFLMSTKYNKDSKMYRALQTVFGERL
FGQGLVSECNYERWHKQRRVIDLAFSRSSLVSLMETFNEKAEQLVEILEAKADGQTPVSM
QDMLTYTAMDILAKAAFGMETSMLLGAQKPLSQAVKLMLEGITASRNTLAKFLPGKRKQL
REVRESIRFLRQVGRDWVQRRREALKRGEEVPADILTQILKAEEGAQDDEGLLDNFVTFF
IAGHETSANHLAFTVMELSRQPEIVARLQAEVDEVIGSKRYLDFEDLGRLQYLSQVLKES
LRLYPPAWGTFRLLEEETLIDGVRVPGNTPLLFSTYVMGRMDTYFEDPLTFNPDRFGPGA
PKPRFTYFPFSLGHRSCIGQQFAQMEVKVVMAKLLQRLEFRLVPGQRFGLQEQATLKPLD
PVLCTLRPRGWQPAPPPPPC
Enzyme 2 Number of Residues 500
Enzyme 2 Molecular Weight 56820.5
Enzyme 2 Theoretical pI 9.38
Enzyme 2 GO Classification
Function
  • binding
  • catalytic activity
  • cation binding
  • electron carrier activity
  • heme binding
  • ion binding
  • iron ion binding
  • metal ion binding
  • monooxygenase activity
  • oxidoreductase activity
  • transition metal ion binding
Process
  • metabolic process
  • oxidation reduction
Component
—
Enzyme 2 General Function Involved in monooxygenase activity
Enzyme 2 Specific Function Involved in the turnover of cholesterol. It converts cholesterol into 24S-hydroxycholesterol and, to a lesser extent, 25-hydroxycholesterol
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions
  • cholesterol + NADPH + H+ + O2 = (24S)-24-hydroxycholesterol + NADP+ + H2O [RN:R07207]
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • 3-23
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 5257114 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q9Y6A2 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name CP46A_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence >1503 bp 
ATGAGCCCCGGGCTGCTGCTGCTCGGCAGCGCCGTCCTGCTCGCCTTCGGCCTCTGCTGC
ACCTTCGTGCACCGCGCTCGCAGCCGCTACGAGCACATCCCCGGGCCGCCGCGGCCCAGT
TTCCTTCTAGGACACCTCCCCTGCTTTTGGAAAAAGGATGAGGTTGGTGGCCGTGTGCTC
CAAGATGTGTTTTTGGATTGGGCTAAGAAGTATGGACCTGTTGTGCGGGTCAACGTCTTC
CACAAAACCTCAGTCATCGTCACGAGTCCTGAGTCGGTTAAGAAGTTCCTGATGTCAACC
AAGTACAACAAGGACTCCAAGATGTACCGTGCGCTCCAGACTGTGTTTGGTGAGAGACTC
TTCGGCCAAGGCTTGGTGTCCGAATGCAACTATGAGCGCTGGCACAAGCAGCGGAGAGTC
ATAGACCTGGCCTTCAGCCGGAGCTCCTTGGTTAGCTTAATGGAAACATTCAACGAGAAG
GCTGAGCAGCTGGTGGAGATTCTAGAAGCCAAGGCAGATGGGCAGACCCCAGTGTCCATG
CAGGACATGCTGACCTACACCGCCATGGACATCCTGGCCAAGGCAGCTTTTGGGATGGAG
ACCAGTATGCTGCTGGGTGCCCAGAAGCCTCTGTCCCAGGCAGTGAAACTTATGTTGGAG
GGAATCACTGCGTCCCGCAACACTCTGGCAAAGTTCCTGCCAGGGAAGAGGAAGCAGCTC
CGGGAGGTCCGGGAGAGCATTCGCTTCCTGCGCCAGGTGGGCAGGGACTGGGTCCAGCGC
CGCCGGGAAGCCCTGAAGAGGGGCGAGGAGGTTCCTGCCGACATCCTCACACAGATTCTG
AAAGCTGAAGAGGGAGCCCAGGACGACGAGGGTCTGCTGGACAACTTCGTCACCTTCTTC
ATTGCTGGTCACGAGACCTCTGCCAACCACTTGGCGTTCACAGTGATGGAGCTGTCTCGC
CAGCCAGAGATCGTGGCAAGGCTGCAGGCCGAGGTGGATGAGGTCATTGGTTCTAAGAGG
TACCTGGATTTCGAGGACCTGGGGAGACTGCAGTACCTGTCCCAGGTCCTCAAAGAGTCG
CTGAGGCTGTACCCACCAGCATGGGGCACCTTTCGCCTGCTGGAAGAGGAGACCTTGATT
GATGGGGTCAGAGTCCCCGGCAACACCCCGCTCTTGTTCAGCACCTATGTCATGGGGCGG
ATGGACACATACTTTGAGGACCCGCTGACTTTCAACCCCGATCGCTTCGGCCCTGGAGCA
CCCAAGCCACGGTTCACCTACTTCCCCTTCTCCCTGGGCCACCGCTCCTGCATCGGGCAG
CAGTTTGCTCAGATGGAGGTGAAGGTGGTCATGGCAAAGCTGCTGCAGAGGCTGGAGTTC
CGGCTGGTGCCCGGGCAGCGCTTCGGGCTGCAGGAGCAGGCCACACTCAAGCCACTGGAC
CCCGTGCTGTGCACCCTGCGGCCCCGCGGCTGGCAGCCCGCACCCCCACCACCCCCCTGC
TGA
Enzyme 2 GenBank Gene ID AF094480 Link Image
Enzyme 2 GeneCard ID CYP46A1 Link Image
Enzyme 2 GenAtlas ID CYP46A1 Link Image
Enzyme 2 HGNC ID HGNC:2641 Link Image
Enzyme 2 Chromosome Location 1
Enzyme 2 Locus 14q32.1
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References
  1. Lund EG, Guileyardo JM, Russell DW: cDNA cloning of cholesterol 24-hydroxylase, a mediator of cholesterol homeostasis in the brain. Proc Natl Acad Sci U S A. 1999 Jun 22;96(13):7238-43. [PubMed Link Image]
  2. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed Link Image]
  3. Mast N, Norcross R, Andersson U, Shou M, Nakayama K, Bjorkhem I, Pikuleva IA: Broad substrate specificity of human cytochrome P450 46A1 which initiates cholesterol degradation in the brain. Biochemistry. 2003 Dec 9;42(48):14284-92. [PubMed Link Image]
Enzyme 2 Metabolite References Not Available