| Common Name |
Malonylcarnitine
| Description |
Malonylcarnitine is a metabolite that accumliates with specific disruption of fatty-acid oxidation caused by impaired entry of long-chain acylcarnitine esters into the mitochondria and failure of the mitochondrial respiratory chain at complex 11 and malonyl-CoA decarboxylase (EC 4.1.1.9) deficiency (OMIM 248360 ). Malonylcarnitine has also been found to accumliate in some newborns with medium-chain acyl-CoA dehydrogenase (EC 1.3.99.3) deficiency (OMIM 201450 ). (PMID 11558490 , 15303003 , 12651823 ).
| Structure |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
2-[(2-Carboxyacetyl)oxy]-4-hydroxy-N,N,N-trimethyl-4-oxo-1-butanaminium inner saltHMDB
3-Carboxy-2-[(carboxyacetyl)oxy]-N,N,N-trimethyl-1-propanaminium inner saltHMDB
Malonyl-L-carnitineHMDB
| Chemical Formlia |
C10H17NO6
| Average Molecliar Weight |
247.2451
| Monoisotopic Molecliar Weight |
247.105587281
| IUPAC Name |
(3S)-3-[(2-carboxyacetyl)oxy]-4-(trimethylazaniumyl)butanoate
| Traditional Name |
(3S)-3-[(2-carboxyacetyl)oxy]-4-(trimethylammonio)butanoate
| CAS Registry Number |
853728-01-5
| SMILES |
C[N+](C)(C)C[C@H](CC([O-])=O)OC(=O)CC(O)=O
| InChI Identifier |
InChI=1S/C10H17NO6/c1-11(2,3)6-7(4-8(12)13)17-10(16)5-9(14)15/h7H,4-6H2,1-3H3,(H-,12,13,14,15)/t7-/m0/s1
| InChI Key |
ZGNBLKBZJBJFDG-ZETCQYMHSA-N
| Chemical Taxonomy |
| Description |
This 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.
| Kingdom |
Chemical entities
| Super Class |
Organic compounds
| Class |
Lipids and lipid-like moleclies
| Sub Class |
Fatty Acyls
| Direct Parent |
Acyl carnitines
| Alternative Parents |
Tricarboxylic acids and derivatives
1,3-dicarbonyl compounds
Tetraalkylammonium salts
Carboxylic acid salts
Carboxylic acid esters
Carboxylic acids
Organopnictogen compounds
Organic salts
Organic oxides
Hydrocarbon derivatives
Amines
| Substituents |
Acyl-carnitine
Tricarboxylic acid or derivatives
1,3-dicarbonyl compound
Quaternary ammonium salt
Tetraalkylammonium salt
Carboxylic acid salt
Carboxylic acid ester
Carboxylic acid
Carboxylic acid derivative
Hydrocarbon derivative
Organic salt
Organopnictogen compound
Organic oxygen compound
Organic nitrogen compound
Organooxygen compound
Organonitrogen compound
Carbonyl group
Amine
Organic oxide
Aliphatic acyclic compound
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
O-acyl-D-carnitine (CHEBI:86047 )
| Ontology |
| Status |
Detected 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 Emlisifiers
| Cellliar locations |
Extracellliar
Membrane
| Physical Properties |
| State |
Solid
| Experimental Properties |
| Property |
Value |
Reference |
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
| Predicted Properties |
| Property |
Value |
Source |
Water Solubility0.34 mg/mLALOGPS
logP-2ALOGPS
logP-4.6ChemAxon
logS-3ALOGPS
pKa (Strongest Acidic)3.47ChemAxon
pKa (Strongest Basic)-7.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area103.73 Å2ChemAxon
Rotatable Bond Count8ChemAxon
Refractivity78.98 m3·mol-1ChemAxon
Polarizability23.61 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
Not Available
| Biological Properties |
| Cellliar Locations |
Extracellliar
Membrane
| Biofluid Locations |
Blood
Urine
| Tissue Location |
Not Available
| Pathways |
Not Available
| Normal Concentrations |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
BloodDetected and Quantified0.1 (0 – 0.2) uMChildren (1-13 years old)Not SpecifiedNormal
11558490
details
UrineDetected and Quantified0.02 (0.01-0.04) umol/mmol creatinineAdlit (>18 years old)Both
Normal
24023812
details
|
| Abnormal Concentrations |
|
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
BloodDetected and Quantified2.7 (1.6 – 3.8) uMNewborn (0-30 days old)Not SpecifiedMalonyl-CoA decarboxylase deficiency
12651823
details
BloodDetected and Quantified0.2 (0.13 – 0.3) uMInfant (0-1 year old)Not SpecifiedMalonyl-CoA decarboxylase deficiency
16078122
details
| Associated Disorders and Diseases |
| Disease References |
| Malonyl-Coa decarboxylase deficiency |
- Santer R, Fingerhut R, Lassker U, Wightman PJ, Fitzpatrick DR, Olgemoller B, Roscher AA: Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency. Clin Chem. 2003 Apr;49(4):660-2. [PubMed:12651823 ]
- Ficicioglu C, Chrisant MR, Payan I, Chace DH: Cardiomyopathy and hypotonia in a 5-month-old infant with malonyl-coa decarboxylase deficiency: potential for preclinical diagnosis with expanded newborn screening. Pediatr Cardiol. 2005 Nov-Dec;26(6):881-3. [PubMed:16078122 ]
| Associated OMIM IDs |
248360 (Malonyl-Coa decarboxylase deficiency)
| External Links |
| DrugBank ID |
Not Available
| DrugBank Metabolite ID |
Not Available
| Phenol Explorer Compound ID |
Not Available
| Phenol Explorer Metabolite ID |
Not Available
| FoodDB ID |
FDB022843
| KNApSAcK ID |
Not Available
| Chemspider ID |
17216162
| KEGG Compound ID |
Not Available
| BioCyc ID |
Not Available
| BiGG ID |
Not Available
| Wikipedia Link |
Not Available
| NuGOwiki Link |
HMDB02095
| Metagene Link |
HMDB02095
| METLIN ID |
6484
| PubChem Compound |
22833583
| PDB ID |
Not Available
| ChEBI ID |
Not Available
Product: Endoxifen (Z-isomer hydrochloride)
References |
| Synthesis Reference |
Not Available |
| Material Safety Data Sheet (MSDS) |
Not Available |
| General References |
- Johnson DW, Trinh MU: Stability of malonylcarnitine and glutarylcarnitine in stored blood spots. J Inherit Metab Dis. 2004;27(6):789-90. [PubMed:15617193 ]
- Santer R, Fingerhut R, Lassker U, Wightman PJ, Fitzpatrick DR, Olgemoller B, Roscher AA: Tandem mass spectrometric determination of malonylcarnitine: diagnosis and neonatal screening of malonyl-CoA decarboxylase deficiency. Clin Chem. 2003 Apr;49(4):660-2. [PubMed:12651823 ]
- Napolitano N, Wiley V, Pitt JJ: Pseudo-glutarylcarnitinaemia in medium-chain acyl-CoA dehydrogenase deficiency detected by tandem mass spectrometry newborn screening. J Inherit Metab Dis. 2004;27(4):465-71. [PubMed:15303003 ]
- Wolf A, Weir P, Segar P, Stone J, Shield J: Impaired fatty acid oxidation in propofol infusion syndrome. Lancet. 2001 Feb 24;357(9256):606-7. [PubMed:11558490 ]
- Ficicioglu C, Chrisant MR, Payan I, Chace DH: Cardiomyopathy and hypotonia in a 5-month-old infant with malonyl-coa decarboxylase deficiency: potential for preclinical diagnosis with expanded newborn screening. Pediatr Cardiol. 2005 Nov-Dec;26(6):881-3. [PubMed:16078122 ]
|
PMID: 20305287