Propionylcarnitine (CHEM003403)

IdentificationTaxonomyBiological PropertiesPhysical PropertiesToxicity ProfileSpectraConcentrationsLinksReferencesXML
Record Information
Version1.0
Creation Date2014-08-29 06:51:18 UTC
Update Date2026-04-03 00:20:24 UTC
Accession NumberCHEM003403
Identification
Common NamePropionylcarnitine
ClassSmall Molecule
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.
Contaminant Sources
  • FooDB Chemicals
  • HMDB Contaminants - Urine
  • T3DB toxins
Contaminant Type
  • Animal Toxin
  • Ester
  • Ether
  • Food Toxin
  • Metabolite
  • Natural Compound
  • Organic Compound
Chemical Structure
Thumb
MOLSDFPDBSMILESInChI
Synonyms
ValueSource
3-Carboxy-N,N,N-trimethyl-2-(1-oxopropoxy)-1-propanaminium inner saltChEBI
O-PropionylcarnitineChEBI
Propionyl carnitineChEBI
(+/-)-propionylcarnitine chlorideHMDB
(3-Carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner saltHMDB
L-PropionylcarnitineHMDB
O-PropanoylcarnitineHMDB
Propionyl-carnitineHMDB
Propionyl-L-carnitineHMDB
Propionylcarnitine, (+-)-isomerHMDB
Propionylcarnitine, (R)-isomerHMDB
Chemical FormulaC10H19NO4
Average Molecular Mass217.262 g/mol
Monoisotopic Mass217.131 g/mol
CAS Registry Number17298-37-2
IUPAC NameNot Available
Traditional Namepropionyl-L-carnitine
SMILESCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI IdentifierInChI=1S/C10H19NO4/c1-5-10(14)15-8(6-9(12)13)7-11(2,3)4/h8H,5-7H2,1-4H3
InChI KeyUFAHZIUFPNSHSL-UHFFFAOYSA-N
Chemical Taxonomy
Description 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
  • Dicarboxylic acid or derivatives
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid ester
  • Carboxylic acid salt
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Biological Properties
StatusDetected and Not Quantified
OriginEndogenous
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
  • Peroxisome
Biofluid LocationsNot Available
Tissue LocationsNot Available
Pathways
NameSMPDB LinkKEGG Link
Oxidation of Branched Chain Fatty AcidsSMP00030 Not Available
Propionic AcidemiaSMP00236 Not Available
Applications
Biological Roles
Chemical RolesNot Available
Physical Properties
StateSolid
AppearanceWhite powder.
Experimental Properties
PropertyValue
Melting PointNot Available
Boiling PointNot Available
SolubilityNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.15 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count7ChemAxon
Refractivity77.26 m³·mol⁻¹ChemAxon
Polarizability22.72 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
SpectraNot Available
Toxicity Profile
Route of ExposureNot Available
Mechanism of ToxicityNot Available
MetabolismNot Available
Toxicity ValuesNot Available
Lethal DoseNot Available
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Uses/SourcesThis is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.
Minimum Risk LevelNot Available
Health EffectsNot Available
SymptomsNot Available
TreatmentNot Available
Concentrations
Not Available
DrugBank IDNot Available
HMDB IDHMDB0000824
FooDB IDFDB022268
Phenol Explorer IDNot Available
KNApSAcK IDNot Available
BiGG ID1862604
BioCyc IDNot Available
METLIN ID5787
PDB IDNot Available
Wikipedia LinkNot Available
Chemspider ID96904
ChEBI ID28867
PubChem Compound ID107738
Kegg Compound IDC03017
YMDB IDNot Available
ECMDB IDNot Available
References
Synthesis ReferenceNot Available
MSDSNot Available
General References
1. https://www.ncbi.nlm.nih.gov/pubmed/?term=22770225
2. Xi X, Kwok LY, Wang Y, Ma C, Mi Z, Zhang H: Ultra-performance liquid chromatography-quadrupole-time of flight mass spectrometry MS(E)-based untargeted milk metabolomics in dairy cows with subclinical or clinical mastitis. J Dairy Sci. 2017 Jun;100(6):4884-4896. doi: 10.3168/jds.2016-11939. Epub 2017 Mar 23.
3. Kurt J. Boudonck, Matthew W. Mitchell, Jacob Wulff and John A. Ryals. Characterization of the biochemical variability of bovine milk using metabolomics. Metabolomics (2009) 5:375?386
4. A. Foroutan et al. The Chemical Composition of Commercial Cow's Milk (in preparation)
5. 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.
6. Penn D, Schmidt-Sommerfeld E, Jakobs C, Bieber LL: Amniotic fluid propionylcarnitine in methylmalonic aciduria. J Inherit Metab Dis. 1987;10(4):376-82.
7. 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.
8. 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.
9. 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.
10. 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.
11. 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.
12. 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.
13. 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.
14. 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.
15. 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.
16. 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.