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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:24 UTC
HMDB IDHMDB00824
Secondary Accession NumbersNone
Metabolite Identification
Common NamePropionylcarnitine
DescriptionPropionylcarnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency, (together with Methylmalonic acid). MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA (OMIM 609058 ).
Structure
Thumb
Synonyms
ValueSource
3-Carboxy-N,N,N-trimethyl-2-(1-oxopropoxy)-1-propanaminium inner saltChEBI
O-PropionylcarnitineChEBI
Propionyl carnitineChEBI
PropionylcarnitineChEBI
3-Propanoyloxy-4-(trimethylazaniumyl)butanoic acidGenerator
(+/-)-propionylcarnitine chlorideHMDB
(3-Carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner saltHMDB
L-PropionylcarnitineHMDB
O-PropanoylcarnitineHMDB
Propionyl-carnitineHMDB
Propionyl-L-carnitineHMDB
Chemical FormulaC10H19NO4
Average Molecular Weight217.2622
Monoisotopic Molecular Weight217.131408101
IUPAC Name3-(propanoyloxy)-4-(trimethylazaniumyl)butanoate
Traditional Namepropionyl-L-carnitine
CAS Registry Number17298-37-2
SMILES
CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI Identifier
InChI=1S/C10H19NO4/c1-5-10(14)15-8(6-9(12)13)7-11(2,3)4/h8H,5-7H2,1-4H3
InChI KeyInChIKey=UFAHZIUFPNSHSL-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acid esters
Direct ParentAcyl carnitines
Alternative Parents
Substituents
  • Acyl-carnitine
  • Carnitine
  • Acyl choline
  • Choline
  • Dicarboxylic acid or derivatives
  • Quaternary ammonium salt
  • Carboxylic acid salt
  • Carboxylic acid ester
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organic salt
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Organic zwitterion
  • 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
  • Lipid catabolism, Fatty acid transport, Energy production
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
  • Peroxisome
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 Solubility0.15 mg/mLALOGPS
logP-2.3ALOGPS
logP-3.7ChemAxon
logS-3.2ALOGPS
pKa (Strongest Acidic)4.19ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area66.43 Å2ChemAxon
Rotatable Bond Count7ChemAxon
Refractivity77.26 m3·mol-1ChemAxon
Polarizability22.72 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
  • Peroxisome
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Oxidation of Branched Chain Fatty AcidsSMP00030Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.31 +/- 0.15 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.400 +/- 0.021 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.564 (0.209-1.159) uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.018 +/- 0.009 uMAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified0.068 +/- 0.054 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.07 (0.01-0.20) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.4814 +/- 0.371 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.247 +/- 0.014 uMAdult (>18 years old)BothCeliac disease details
UrineDetected and Quantified0.3638 +/- 0.6673 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.218 +/- 0.2137 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
Celiac disease
  1. Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [16425363 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022268
KNApSAcK IDNot Available
Chemspider ID96904
KEGG Compound IDC03017
BioCyc IDNot Available
BiGG ID1862604
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00824
Metagene LinkHMDB00824
METLIN ID5787
PubChem Compound107738
PDB IDNot Available
ChEBI ID28867
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Roe CR, Struys E, Kok RM, Roe DS, Harris RA, Jakobs C: Methylmalonic semialdehyde dehydrogenase deficiency: psychomotor delay and methylmalonic aciduria without metabolic decompensation. Mol Genet Metab. 1998 Sep;65(1):35-43. [9787093 ]
  2. Ferrara F, Bertelli A, Falchi M: Evaluation of carnitine, acetylcarnitine and isovalerylcarnitine on immune function and apoptosis. Drugs Exp Clin Res. 2005;31(3):109-14. [16033249 ]
  3. Vermeulen RC, Scholte HR: Exploratory open label, randomized study of acetyl- and propionylcarnitine in chronic fatigue syndrome. Psychosom Med. 2004 Mar-Apr;66(2):276-82. [15039515 ]
  4. Vernez L, Wenk M, Krahenbuhl S: Determination of carnitine and acylcarnitines in plasma by high-performance liquid chromatography/electrospray ionization ion trap tandem mass spectrometry. Rapid Commun Mass Spectrom. 2004;18(11):1233-8. [15164354 ]
  5. Golan R, Soffer Y, Katz S, Weissenberg R, Wasserzug O, Lewin LM: Carnitine and short-chain acylcarnitines in the lumen of the human male reproductive tract. Int J Androl. 1983 Aug;6(4):349-57. [6618689 ]
  6. Matsumoto K, Takahashi M, Takiyama N, Misaki H, Matsuo N, Murano S, Yuki H: Enzyme reactor for urinary acylcarnitines assay by reversed-phase high-performance liquid chromatography. Clin Chim Acta. 1993 Jul 16;216(1-2):135-43. [8222264 ]
  7. Leonard JV, Vijayaraghavan S, Walter JH: The impact of screening for propionic and methylmalonic acidaemia. Eur J Pediatr. 2003 Dec;162 Suppl 1:S21-4. Epub 2003 Oct 30. [14586648 ]
  8. Penn D, Schmidt-Sommerfeld E, Jakobs C, Bieber LL: Amniotic fluid propionylcarnitine in methylmalonic aciduria. J Inherit Metab Dis. 1987;10(4):376-82. [3126357 ]
  9. Di Donato S, Rimoldi M, Garavaglia B, Uziel G: Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency. Clin Chim Acta. 1984 May 16;139(1):13-21. [6723070 ]
  10. Bertelli A, Conte A, Ronca G: L-propionyl carnitine protects erythrocytes and low density lipoproteins against peroxidation. Drugs Exp Clin Res. 1994;20(5):191-7. [7875055 ]
  11. Van Hove JL, Chace DH, Kahler SG, Millington DS: Acylcarnitines in amniotic fluid: application to the prenatal diagnosis of propionic acidaemia. J Inherit Metab Dis. 1993;16(2):361-7. [8411998 ]
  12. Lucke T, Perez-Cerda C, Baumgartner M, Fowler B, Sander S, Sasse M, Scholl S, Ugarte M, Das AM: Propionic acidemia: unusual course with late onset and fatal outcome. Metabolism. 2004 Jun;53(6):809-10. [15164333 ]

Enzymes

General function:
Involved in acyltransferase activity
Specific function:
Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of acetyl-CoA into mitochondria.
Gene Name:
CRAT
Uniprot ID:
P43155
Molecular weight:
70875.095
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