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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-05-22 14:17:49 UTC
Update Date2018-05-20 08:56:41 UTC
HMDB IDHMDB0002356
Secondary Accession Numbers
  • HMDB0060125
  • HMDB02356
  • HMDB60125
Metabolite Identification
Common NameHexacosanoic acid
DescriptionX-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder biochemically characterized by the accumulation of very long chain fatty acids (VLCFA), particularly hexacosanoic acid (C(26:0)) and tetracosanoic acid (C(24:0)), in tissues and biological fluids. (PMID 16750542 ).
Structure
Thumb
Synonyms
ValueSource
C26:0ChEBI
Ceratinic acidChEBI
Ceric acidChEBI
Cerinic acidChEBI
Cerotic acidChEBI
Cerylic acidChEBI
CH3-[CH2]24-COOHChEBI
HexacosansaeureChEBI
Hexacosoic acidChEBI
Hexaeicosanoic acidChEBI
N-C26:0ChEBI
N-Hexacosanoic acidChEBI
HexacosanoateGenerator
CeratinateGenerator
CerateGenerator
CerinateGenerator
CerotateGenerator
CerylateGenerator
HexacosoateGenerator
HexaeicosanoateGenerator
N-HexacosanoateGenerator
Hexacosanoate (N-C26:0)HMDB
Chemical FormulaC26H52O2
Average Molecular Weight396.6899
Monoisotopic Molecular Weight396.396730908
IUPAC Namehexacosanoic acid
Traditional Namehexacosanoic acid
CAS Registry Number506-46-7
SMILES
CCCCCCCCCCCCCCCCCCCCCCCCCC(O)=O
InChI Identifier
InChI=1S/C26H52O2/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-22-23-24-25-26(27)28/h2-25H2,1H3,(H,27,28)
InChI KeyXMHIUKTWLZUKEX-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as very long-chain fatty acids. These are fatty acids with an aliphatic tail that contains at least 22 carbon atoms.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentVery long-chain fatty acids
Alternative Parents
Substituents
  • Very long-chain fatty acid
  • Straight chain fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point88.5 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility1.8e-05 g/LALOGPS
logP9.87ALOGPS
logP10.7ChemAxon
logS-7.3ALOGPS
pKa (Strongest Acidic)4.95ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count2ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area37.3 ŲChemAxon
Rotatable Bond Count24ChemAxon
Refractivity123.09 m³·mol⁻¹ChemAxon
Polarizability55.83 ųChemAxon
Number of Rings0ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-0159-2900100000-dc0324482e5853e76290View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0159-2900100000-dc0324482e5853e76290View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0159-2900100000-dc0324482e5853e76290View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0159-1900000000-9d83fa08f63878dd9794View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-8890000000-a358b076f9e4d3b08976View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-0fmr-9550000000-f90d4cc6a83ff8e16245View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0a4j-0109000000-bc2c74027165503fd92fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0pb9-2193000000-cf0ac4a96f99022c7b3aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0w30-6096000000-65025824de97c5fb5ff7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-002b-0009000000-bd3c909a0431684b20e7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0ug1-3439000000-ca5336c838aa14069626View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000f-6974000000-93ebd8f603ed3f48dd9dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-0009000000-50e9c7751bdac41b78c8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0f6t-0009000000-fdc9aa019c323235312eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9113000000-af814ec2317a697df1a0View in MoNA
MSMass Spectrum (Electron Ionization)splash10-0597-9201000000-41113a5b904c5db7332dView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
Tissue Locations
  • Adrenal Gland
  • Fibroblasts
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.110 +/- 0.006 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<0.9 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified0.832 +/- 0.454 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified0.832 +/- 0.454 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified0.45-1.32 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified0.83 +/- 0.45 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.781 (0.277-1.563) uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified0.790 (0.450-1.320) uMNot SpecifiedBothNormal
    • Physician's Guide...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.0-1.3 uMChildren (1-13 years old)MaleMental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma details
BloodDetected and Quantified0.993 (0.252-1.622) uMNewborn (0-30 days old)BothAdrenoleukodystrophy, neonatal
    • MetaGene: Metabol...
details
BloodDetected and Quantified6.806 uMInfant (0-1 year old)MalePseudo Zellweger->D-bifunctional protein deficiency details
BloodDetected and Quantified7.167 uMChildren (1-13 years old)FemalePseudoneonatal adrenoleukodystrophy details
BloodDetected and Quantified2.571-4.512 uMAdult (>18 years old)FemaleX-linked adrenoleukodystrophy details
BloodDetected and Quantified2.26 uMChildren (1-13 years old)Not SpecifiedX-linked adrenoleukodystrophy details
BloodDetected and Quantified5.4 uMInfant (0-1 year old)Not SpecifiedD-Bifunctional Protein Deficiency details
BloodDetected and Quantified1.21 uMInfant (0-1 year old)Male
Peroxisomal biogenesis disorder
details
BloodDetected and Quantified1.33 uMNewborn (0-30 days old)Female
Rhizomelic chondrodysplasia punctata
details
BloodDetected and Quantified4.10 (2.42 - 6.43) uMAdult (>18 years old)BothAdrenomyeloneuropathy details
BloodDetected and Quantified7.9 uMNewborn (0-30 days old)Not Specified
Zellweger syndrome
details
BloodDetected and Quantified1.300-2.370 uMChildren (1-13 years old)Female
Peroxisomal biogenesis disorder
details
BloodDetected and Quantified2.0167-2.0923 uMChildren (1-13 years old)Male
Peroxisomal disorders, new type, liver
details
BloodDetected and Quantified4.710-6.700 uMNewborn (0-30 days old)Female
Peroxisomal biogenesis disorder
details
BloodDetected and Quantified7.200-18.400 uMNewborn (0-30 days old)Not Specified
Neonatal adrenoleukodystrophy
details
BloodDetected and Quantified7.310 (4.185-11.218) uMChildren (1-13 years old)Both
Zellweger syndrome
details
Associated Disorders and Diseases
Disease References
Adrenoleukodystrophy
  1. Steinberg SJ, Fensom AH, Dalton NR, Toseland PA, Kennedy CR, Mowat AP: Measurement of plasma very long-chain fatty acids as a preliminary screening procedure for the diagnosis of peroxisomal disorders. J Inherit Metab Dis. 1994;17(3):323-6. [PubMed:7807943 ]
  2. ten Brink HJ, van den Heuvel CM, Poll-The BT, Wanders RJ, Jakobs C: Peroxisomal disorders: concentrations of metabolites in cerebrospinal fluid compared with plasma. J Inherit Metab Dis. 1993;16(3):587-90. [PubMed:7609459 ]
Adrenomyeloneuropathy
  1. Sadeghi-Nejad A, Senior B: Adrenomyeloneuropathy presenting as Addison's disease in childhood. N Engl J Med. 1990 Jan 4;322(1):13-6. [PubMed:2294415 ]
D-Bifunctional protein deficiency
  1. Watkins PA, Chen WW, Harris CJ, Hoefler G, Hoefler S, Blake DC Jr, Balfe A, Kelley RI, Moser AB, Beard ME, et al.: Peroxisomal bifunctional enzyme deficiency. J Clin Invest. 1989 Mar;83(3):771-7. [PubMed:2921319 ]
  2. Rizzo C, Boenzi S, Wanders RJ, Duran M, Caruso U, Dionisi-Vici C: Characteristic acylcarnitine profiles in inherited defects of peroxisome biogenesis: a novel tool for screening diagnosis using tandem mass spectrometry. Pediatr Res. 2003 Jun;53(6):1013-8. doi: 10.1203/01.PDR.0000064902.59052.0F. Epub 2003 Mar 19. [PubMed:12646728 ]
Peroxisomal biogenesis defect
  1. Baumgartner MR, Poll-The BT, Verhoeven NM, Jakobs C, Espeel M, Roels F, Rabier D, Levade T, Rolland MO, Martinez M, Wanders RJ, Saudubray JM: Clinical approach to inherited peroxisomal disorders: a series of 27 patients. Ann Neurol. 1998 Nov;44(5):720-30. [PubMed:9818927 ]
  2. Mandel H, Espeel M, Roels F, Sofer N, Luder A, Iancu TC, Aizin A, Berant M, Wanders RJ, Schutgens RB: A new type of peroxisomal disorder with variable expression in liver and fibroblasts. J Pediatr. 1994 Oct;125(4):549-55. [PubMed:7931872 ]
Rhizomelic chondrodysplasia punctata
  1. Baumgartner MR, Poll-The BT, Verhoeven NM, Jakobs C, Espeel M, Roels F, Rabier D, Levade T, Rolland MO, Martinez M, Wanders RJ, Saudubray JM: Clinical approach to inherited peroxisomal disorders: a series of 27 patients. Ann Neurol. 1998 Nov;44(5):720-30. [PubMed:9818927 ]
Peroxisomal disorders, new type, liver
  1. Mandel H, Espeel M, Roels F, Sofer N, Luder A, Iancu TC, Aizin A, Berant M, Wanders RJ, Schutgens RB: A new type of peroxisomal disorder with variable expression in liver and fibroblasts. J Pediatr. 1994 Oct;125(4):549-55. [PubMed:7931872 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Adrenoleukodystrophy, neonatal
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Mental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma
  1. Martinelli D, Travaglini L, Drouin CA, Ceballos-Picot I, Rizza T, Bertini E, Carrozzo R, Petrini S, de Lonlay P, El Hachem M, Hubert L, Montpetit A, Torre G, Dionisi-Vici C: MEDNIK syndrome: a novel defect of copper metabolism treatable by zinc acetate therapy. Brain. 2013 Mar;136(Pt 3):872-81. doi: 10.1093/brain/awt012. Epub 2013 Feb 18. [PubMed:23423674 ]
Pseudoneonatal adrenoleukodystrophy
  1. Poll-The BT, Roels F, Ogier H, Scotto J, Vamecq J, Schutgens RB, Wanders RJ, van Roermund CW, van Wijland MJ, Schram AW, et al.: A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy). Am J Hum Genet. 1988 Mar;42(3):422-34. [PubMed:2894756 ]
Associated OMIM IDs
  • 300100 (Adrenomyeloneuropathy)
  • 215100 (Rhizomelic chondrodysplasia punctata)
  • 214100 (Peroxisomal biogenesis defect)
  • 300100 (Adrenoleukodystrophy)
  • 261515 (D-Bifunctional protein deficiency)
  • 264470 (Pseudoneonatal adrenoleukodystrophy)
  • 609313 (Mental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB003045
KNApSAcK IDC00035114
Chemspider ID10037
KEGG Compound IDNot Available
BioCyc IDCPD-7832
BiGG ID1459812
Wikipedia LinkCerotic acid
METLIN ID6642
PubChem Compound10469
PDB IDNot Available
ChEBI ID31009
References
Synthesis ReferenceTsuji S; Sano T; Ariga T; Miyatake T Increased synthesis of hexacosanoic acid (C23:0) by cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN). Journal of biochemistry (1981), 90(4), 1233-6.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Dhaunsi GS, Kaur J, Alsaeid K, Turner RB, Bitar MS: Very long chain fatty acids activate NADPH oxidase in human dermal fibroblasts. Cell Biochem Funct. 2005 Jan-Feb;23(1):65-8. [PubMed:15565636 ]
  2. Moser HW, Moser AB, Powers JM, Nitowsky HM, Schaumburg HH, Norum RA, Migeon BR: The prenatal diagnosis of adrenoleukodystrophy. Demonstration of increased hexacosanoic acid levels in cultured amniocytes and fetal adrenal gland. Pediatr Res. 1982 Mar;16(3):172-5. [PubMed:7063272 ]
  3. Thiele I, Swainston N, Fleming RM, Hoppe A, Sahoo S, Aurich MK, Haraldsdottir H, Mo ML, Rolfsson O, Stobbe MD, Thorleifsson SG, Agren R, Bolling C, Bordel S, Chavali AK, Dobson P, Dunn WB, Endler L, Hala D, Hucka M, Hull D, Jameson D, Jamshidi N, Jonsson JJ, Juty N, Keating S, Nookaew I, Le Novere N, Malys N, Mazein A, Papin JA, Price ND, Selkov E Sr, Sigurdsson MI, Simeonidis E, Sonnenschein N, Smallbone K, Sorokin A, van Beek JH, Weichart D, Goryanin I, Nielsen J, Westerhoff HV, Kell DB, Mendes P, Palsson BO: A community-driven global reconstruction of human metabolism. Nat Biotechnol. 2013 May;31(5):419-25. doi: 10.1038/nbt.2488. Epub 2013 Mar 3. [PubMed:23455439 ]
  4. Deon M, Wajner M, Sirtori LR, Fitarelli D, Coelho DM, Sitta A, Barschak AG, Ferreira GC, Haeser A, Giugliani R, Vargas CR: The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy. J Neurol Sci. 2006 Sep 25;247(2):157-64. Epub 2006 Jun 5. [PubMed:16750542 ]

Enzymes

General function:
Involved in catalytic activity
Specific function:
Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses palmitoleate, oleate and linoleate.
Gene Name:
ACSL1
Uniprot ID:
P33121
Molecular weight:
77942.685