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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2007-05-22 16:35:51 UTC
Update Date2017-10-23 19:04:19 UTC
HMDB IDHMDB0006202
Secondary Accession Numbers
  • HMDB06202
Metabolite Identification
Common Name4,8 Dimethylnonanoyl carnitine
Description4,8 dimethylnonanoyl carnitine is an intermediate in phytanic and pristanic acid metabolism. Both phytanic acid and pristanic acid are initially oxidized in peroxisomes to 4,8-dimethylnonanoyl-CoA, which is then converted to to 4,8-dimethylnonanoyl carnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. After transport across the mitochondrial membrane and transfer of the acylgroup to coenzyme A, further oxidation to 2,6-dimethylheptanoyl-CoA occurs (PMID: 9469587 ). 4,8 dimethylnonanoyl carnitine is not a substrate for carnitine acetyltransferase, another acyltransferase localized in peroxisomes, which catalyzes the formation of carnitine esters of the other products of pristanic acid beta-oxidation, namely acetyl-CoA and propionyl-CoA. (PMID: 10486279 ). Earlier studies have shown that pristanic acid undergoes three cycles of beta-oxidation in peroxisomes to produce 4,8-dimethylnonanoyl-CoA (DMN-CoA) which is then transported to the mitochondria for full oxidation to CO(2) and H(2)O. In principle, this can be done via two different mechanisms in which DMN-CoA is either converted into the corresponding carnitine ester or hydrolyzed to 4,8-dimethylnonanoic acid plus CoASH.(PMID: 11785945 ). Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) and pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) are branched-chain fatty acids that are constituents of the human diet. As phytanic acid possesses a beta-methyl group, it cannot be degraded by beta-oxidation. Instead, phytanic acid is first degraded by alpha-oxidation, yielding pristanic acid, which is subsequently degraded by beta-oxidation. Phytanic acid alpha-oxidation is thought to occur partly, and pristanic acid beta-oxidation exclusively, in peroxisomes. Accumulation of phytanic acid and pristanic acid is found in blood and tissues of patients affected with generalized peroxisomal disorders.
Structure
Thumb
Synonyms
ValueSource
4,8-DimethylnonanoylcarnitineChEBI
Chemical FormulaC18H35NO4
Average Molecular Weight329.4748
Monoisotopic Molecular Weight329.256608613
IUPAC Name3-[(4,8-dimethylnonanoyl)oxy]-4-(trimethylazaniumyl)butanoate
Traditional Name4,8-dimethylnonanoylcarnitine
CAS Registry NumberNot Available
SMILES
CC(C)CCCC(C)CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C18H35NO4/c1-14(2)8-7-9-15(3)10-11-18(22)23-16(12-17(20)21)13-19(4,5)6/h14-16H,7-13H2,1-6H3
InChI KeyDDTDJDZHDFMZED-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentAcyl carnitines
Alternative Parents
Substituents
  • Acyl-carnitine
  • Branched fatty acid
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic nitrogen compound
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Organooxygen compound
  • Organic salt
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

    Chemical reaction:

    Biochemical process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.00024 g/LALOGPS
logP-0.61ALOGPS
logP-0.5ChemAxon
logS-6.2ALOGPS
pKa (Strongest Acidic)4.29ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 ŲChemAxon
Rotatable Bond Count13ChemAxon
Refractivity113.97 m³·mol⁻¹ChemAxon
Polarizability38.58 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00di-9300000000-7404eec33724333cbe3bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01qc-0925000000-45565d6ef3b2d942da8cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0006-2900000000-d0e0f6f815e417182179View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0abc-9600000000-d22566bfbdfdf194d9e0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-1319000000-298fff1a97503d7e7ca0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0170-1973000000-b1cfe17300810f8c9ed5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0673-4900000000-0babf4d8840191a63c64View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
  • Peroxisome
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB/PathwhizKEGG
Oxidation of Branched Chain Fatty AcidsPw000155Pw000155 greyscalePw000155 simpleNot Available
Displaying 1 entry
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB023834
KNApSAcK IDNot Available
Chemspider ID21233922
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG ID2365396
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound53477801
PDB IDNot Available
ChEBI ID63874
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Ferdinandusse S, Mulders J, IJlst L, Denis S, Dacremont G, Waterham HR, Wanders RJ: Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids. Biochem Biophys Res Commun. 1999 Sep 16;263(1):213-8. [PubMed:10486279 ]
  2. Verhoeven NM, Roe DS, Kok RM, Wanders RJ, Jakobs C, Roe CR: Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts. J Lipid Res. 1998 Jan;39(1):66-74. [PubMed:9469587 ]
  3. Ofman R, el Mrabet L, Dacremont G, Spijer D, Wanders RJ: Demonstration of dimethylnonanoyl-CoA thioesterase activity in rat liver peroxisomes followed by purification and molecular cloning of the thioesterase involved. Biochem Biophys Res Commun. 2002 Jan 18;290(2):629-34. [PubMed:11785945 ]

Enzymes

General function:
Involved in acyltransferase activity
Specific function:
Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate. Converts the end product of pristanic acid beta oxidation, 4,8-dimethylnonanoyl-CoA, to its corresponding carnitine ester.
Gene Name:
CROT
Uniprot ID:
Q9UKG9
Molecular weight:
10213.63
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT2
Uniprot ID:
P23786
Molecular weight:
73776.335
General function:
Involved in transporter activity
Specific function:
Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.
Gene Name:
SLC25A20
Uniprot ID:
O43772
Molecular weight:
32943.46