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Record Information
Version3.6
Creation Date2006-05-22 14:17:49 UTC
Update Date2016-05-13 21:36:27 UTC
HMDB IDHMDB02356
Secondary Accession Numbers
  • 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
Cerylic acidChEBI
CH3-[CH2]24-COOHChEBI
HexacosansaeureChEBI
Hexacosoic acidChEBI
Hexaeicosanoic acidChEBI
N-C26:0ChEBI
N-Hexacosanoic acidChEBI
CerotateGenerator
CeratinateGenerator
CerateGenerator
CerinateGenerator
CerylateGenerator
HexacosoateGenerator
HexaeicosanoateGenerator
N-HexacosanoateGenerator
HexacosanoateHMDB
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 KeyInChIKey=XMHIUKTWLZUKEX-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
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
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 Solubility1.81e-05 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count24ChemAxon
Refractivity123.09 m3·mol-1ChemAxon
Polarizability55.83 Å3ChemAxon
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
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
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, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
MSMass Spectrum (Electron Ionization)splash10-0597-9201000000-41113a5b904c5db7332dView in MoNA
1D NMR1H NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
1D NMR13C NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Adrenal Gland
  • Fibroblasts
Pathways
NameSMPDB LinkKEGG Link
Adrenoleukodystrophy, X-linkedSMP00516Not Available
Beta Oxidation of Very Long Chain Fatty AcidsSMP00052map01040
Carnitine-acylcarnitine translocase deficiencySMP00517Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.110 +/- 0.006 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.83 +/- 0.45 uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.993 (0.252-1.622) uMNewborn (0-30 days old)BothAdrenoleukodystrophy, neonatal details
BloodDetected and Quantified9.93 (2.52-16.22) uMNewborn (0-30 days old)BothNeonatal adrenoleukodystrophy details
BloodDetected and Quantified2.0 (2.0-2.09) uMChildren (1-13 years old)BothPeroxisomal disorders, new type, liver details
BloodDetected and Quantified4.10 (2.42 - 6.43) uMAdult (>18 years old)BothAdrenomyeloneuropathy details
Associated Disorders and Diseases
Disease References
Adrenoleukodystrophy
  1. MetaGene [Link]
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. [2294415 ]
Peroxisomal disorders, new type, liver
  1. MetaGene [Link]
Adrenoleukodystrophy, neonatal
  1. MetaGene [Link]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB003045
KNApSAcK IDC00035114
Chemspider ID10037
KEGG Compound IDNot Available
BioCyc IDCPD-7832
BiGG ID1459812
Wikipedia LinkCerate
NuGOwiki LinkHMDB02356
Metagene LinkHMDB02356
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. [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. [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 Mar 3. doi: 10.1038/nbt.2488. [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. [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