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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 10:11:00 UTC
HMDB IDHMDB0000062
Secondary Accession Numbers
  • HMDB0001467
  • HMDB00062
  • HMDB01467
Metabolite Identification
Common NameL-Carnitine
DescriptionCarnitine is not an essential amino acid; it can be synthesized in the body. However, it is so important in providing energy to muscles (including the heart) that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat (the main food source for carnitine). Carnitine has been described as a vitamin, an amino acid, or a metabimin (i.e. an essential metabolite). Like the B vitamins, carnitine contains nitrogen and is very soluble in water, and to some researchers carnitine is a vitamin (Liebovitz 1984). It was found that an animal (yellow mealworm) could not grow without carnitine in its diet. However, as it turned out, almost all other animals, including humans, do make their own carnitine; thus, it is no longer considered a vitamin. Nevertheless, in certain circumstances, such as deficiencies of methionine, lysine, or vitamin C or kidney dialysis, carnitine shortages develop. Under these conditions, carnitine must be absorbed from food, and for this reason, it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. Like the other amino acids used or manufactured by the body, carnitine is an amine. But like choline, which is sometimes considered to be a B vitamin, carnitine is also an alcohol (specifically, a trimethylated carboxy-alcohol). Thus, carnitine is an unusual amino acid and has different functions than most other amino acids, which are usually employed by the body in the construction of protein. Carnitine is an essential factor in fatty acid metabolism in mammals. Its most important known metabolic function is to transport fat into the mitochondria of muscle cells, including those in the heart, for oxidation. This is how the heart gets most of its energy. In humans, about 25% of carnitine is synthesized in the liver, kidney, and brain from the amino acids lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reye's syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Borum et al. (1979) describe carnitine as an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309 ). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic or organic aciduria (acid urine resulting from genetic or other anomalies), carnitine is essential to life and carnitine supplements are valuable. Carnitine therapy may also be useful in a wide variety of clinical conditions. Carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. It may also be worth a trial for patients with any form of hyperlipidemia or muscle weakness. Carnitine supplements may also be useful in many forms of toxic or metabolic liver disease and in cases of heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine joins a long list of nutrients which may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to the low motility of sperm. Carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia (http://www.dcnutrition.com). Carnitine is a biomarker for the consumption of meat.
Structure
Thumb
Synonyms
ValueSource
(-)-CarnitineChEBI
(-)-L-CarnitineChEBI
3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium hydroxide, inner saltChEBI
CarnicorChEBI
CarniteneChEBI
CarnitineChEBI
CarnitorChEBI
KarnitinChEBI
LevocarnitineChEBI
Vitamin BTChEBI
(-)-(R)-3-Hydroxy-4-(trimethylammonio)butyrateHMDB
(R)-(3-Carboxy-2-hydroxypropyl)trimethylammonium hydroxideHMDB
(R)-CarnitineHMDB
(S)-CarnitineHMDB
1-CarnitineHMDB
3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminiumHMDB
3-Hydroxy-4-trimethylammoniobutanoateHMDB
3-Hydroxy-4-trimethylammoniobutanoic acidHMDB
BicarnesineHMDB
CarnikingHMDB
Carniking 50HMDB
CarnileanHMDB
CarnipassHMDB
Carnipass 20HMDB
D-CarnitineHMDB
delta-CarnitineHMDB
DL-CarnitineHMDB
gamma-Trimethyl-ammonium-beta-hydroxybutirateHMDB
gamma-Trimethyl-beta-hydroxybutyrobetaineHMDB
gamma-Trimethyl-hydroxybutyrobetaineHMDB
L-(-)-CarnitineHMDB
L-gamma-Trimethyl-beta-hydroxybutyrobetaineHMDB
LevocarnitinaHMDB
LevocarnitinumHMDB
R-(-)-3-Hydroxy-4-trimethylaminobutyrateHMDB
L CarnitineMeSH
Chemical FormulaC7H15NO3
Average Molecular Weight161.1989
Monoisotopic Molecular Weight161.105193351
IUPAC Name(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate
Traditional NameL-carnitine
CAS Registry Number541-15-1
SMILES
C[N+](C)(C)C[C@H](O)CC([O-])=O
InChI Identifier
InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1
InChI KeyPHIQHXFUZVPYII-ZCFIWIBFSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as carnitines. These are organic compounds containing the quaternary ammonium compound carnitine.
KingdomOrganic compounds
Super ClassOrganic nitrogen compounds
ClassOrganonitrogen compounds
Sub ClassQuaternary ammonium salts
Direct ParentCarnitines
Alternative Parents
Substituents
  • Carnitine
  • Beta-hydroxy acid
  • Short-chain hydroxy acid
  • Fatty acid
  • Hydroxy acid
  • Tetraalkylammonium salt
  • 1,2-aminoalcohol
  • Carboxylic acid salt
  • Secondary alcohol
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic oxygen compound
  • Organooxygen compound
  • Organic zwitterion
  • Organic salt
  • Hydrocarbon derivative
  • Organic oxide
  • Carbonyl group
  • Organopnictogen compound
  • Amine
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point197 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility5.33 g/LALOGPS
logP-2.9ALOGPS
logP-4.9ChemAxon
logS-1.6ALOGPS
pKa (Strongest Acidic)4.2ChemAxon
pKa (Strongest Basic)-3.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area60.36 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity63.49 m³·mol⁻¹ChemAxon
Polarizability16.93 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-9100000000-1b25dacb04c3ed5be8d0View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9100000000-c15eaa190d1e28a4839aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-03di-0900000000-9b579c570aab7c7d3a21View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0ik9-0900000000-e37d3def2de1af5bd2f5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0ue9-8900000000-cf37f0a721cd52d86e8eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0r0c-9200000000-a11a4872036f3c33d25aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0a4l-9000000000-2402e260a330ff1772a1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , positivesplash10-0w29-6900000000-d0136248acdd706e650bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0ik9-2900000000-61732bb10307f2b183caView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0zg0-9400000000-15c3db3324f1d46d3c7cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0ik9-2900000000-ee79f10dce9fc09246ecView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01ox-0900000000-3bffd5143cf8b072299eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00kf-0900000000-e68f553bcb15d3ef5b47View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00di-9200000000-c5b43723951af6da48cfView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-1900000000-dd1b1a023937cdcb11afView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-114i-3900000000-9f24d3bc535736149e2aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-632ef17d9dde38586990View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Adipose Tissue
  • Bladder
  • Brain
  • Erythrocyte
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Lung
  • Muscle
  • Myocardium
  • Nerve Cells
  • Neuron
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Sperm
  • Testes
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified29.74 +/- 7.55 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified25-88 uMNot SpecifiedNot Specified
Normal
details
BloodDetected and Quantified28.500-109 uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified25.4-54.1 uMAdult (>18 years old)Female
Normal
details
BloodDetected and Quantified40-60 uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified26-66 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified28-47 uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified26-66 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified37-58 uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified50 +/- 30 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified>25 uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified26-70 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified33.6 +/- 6.2 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified38.2 +/- 5.4 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified15.5 (13.2-17.7) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified16.28 +/- 8.41 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified14.60 +/- 5.43 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified43.0 (26.0-79.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified41.2 (35.5-47.5) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified43.57(9.97) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified39.33(13.25) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified40.430 +/- 3.650 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified19.0-65.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified35-75 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified35.3 +/- 7.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified45.7 +/- 11.6 uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified26.2 +/- 10.1 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified1.900 +/- 0.474 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.0 (2.0 - 9.0) uMAdult (>18 years old)BothNormal details
FecesDetected and Quantified19.87 +/- 11.39 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified8.13 +/- 3.11 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified5.24 +/- 1.035 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified0.924 +/- 0.960 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified0.656 +/- 0.734 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
UrineDetected but not Quantified Adult (>18 years old)MaleNormal details
UrineDetected and Quantified6926.20 +/- 754.66 umol/mmol creatinineAdolescent (13-18 years old)Both
Normal
details
UrineDetected and Quantified10.733 +/- 1.733 umol/mmol creatinineAdult (>18 years old)Not Specified
Normal
details
UrineDetected and Quantified3.800-5.133 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
details
UrineDetected and Quantified0.317-40.928 umol/mmol creatinineAdult (>18 years old)Female
Normal
details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected and Quantified4.5 (0.62-15.2) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified5.0 (0.7-16.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified3.17 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified5.7 +/- 4.8 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified18.159 +/- 16.442 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified<100 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified23.5 +/- 4.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified18.0 +/- 1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected but not Quantified Adult (>18 years old)Male
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified22000-50000 uMNot SpecifiedNot Specified3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency details
BloodDetected and Quantified41.7 +/- 23.9 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified17 uMAdult (>18 years old)Male
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
details
BloodDetected and Quantified47-68 uMInfant (0-1 year old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified20.6-33.8 uMAdult (>18 years old)Female
Myopathic carnitine deficiency
details
BloodDetected and Quantified0-2.2 uMInfant (0-1 year old)Female
Long-chain Fatty Acids, Defect in Transport of
details
BloodDetected and Quantified12 uMNewborn (0-30 days old)Male
Carnitine palmitoyltransferase deficiency I
details
BloodDetected and Quantified0 uMInfant (0-1 year old)Female
Carnitine transporter defect; primary systemic carnitine deficiency
details
BloodDetected and Quantified15 uMNewborn (0-30 days old)Male
Carnitine palmitoyltransferase deficiency I
details
BloodDetected and Quantified37-48 uMInfant (0-1 year old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified26.04 +/- 10.5 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified23-72 uMChildren (1-13 years old)Both
Methylmalonic aciduria mitochondrial encephelopathy Leigh-like
details
BloodDetected and Quantified15-34 uMChildren (1-13 years old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified24.3 +/- 11.7 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified9-33 uMAdolescent (13-18 years old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified19-31 uMAdult (>18 years old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified30.2 (6.3) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified10-21 uMInfant (0-1 year old)Not Available
Mitochondrial trifunctional protein deficiency
details
BloodDetected and Quantified31.8 (14.1) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified0.7 uMInfant (0-1 year old)Female
Carnitine transporter defect; primary systemic carnitine deficiency
details
BloodDetected and Quantified4.2 uMChildren (1-13 years old)Male
Carnitine transporter defect; primary systemic carnitine deficiency
details
BloodDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified43-51 uMInfant (0-1 year old)Male
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified16.798 (14.323) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified15.08 +/- 5.71 uMChildren (1-13 years old)Both
Acetaminophen overdose
details
BloodDetected and Quantified21.08 (4.88) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified19.98 (4.82) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified46.8 (24.8) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified18-22 uMInfant (0-1 year old)Male2,4-dienoyl-CoA reductase deficiency details
BloodDetected and Quantified27.8 (20.0) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified50.65(14.14) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified47.22(15.19) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
BloodDetected and Quantified54.38(13.17) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected and Quantified50.38(8.11) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected and Quantified20.540 +/- 1.850 uMChildren (1-13 years old)Not Specified
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
details
BloodDetected and Quantified23-50 uMChildren (1-13 years old)Both
L-2-hydroxyglutaric aciduria
details
BloodDetected and Quantified34.0 +/- 8.8 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified36.2 +/- 8.1 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified25.5 (9.7) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified19.5 (8.3) uMAdult (>18 years old)FemalePregnancy details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
SalivaDetected but not Quantified Adult (>18 years old)BothOral cancer details
SalivaDetected but not Quantified Adult (>18 years old)FemaleBreast cancer details
SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPeriodontal diseases details
SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPancreatic cancer details
UrineDetected and Quantified13.3 (11.6-15.1) umol/mmol creatinineAdult (>18 years old)Both
Diabetes
details
UrineDetected and Quantified41.3 (38.3-44.2) umol/mmol creatinineAdult (>18 years old)Both
Diabetes
details
UrineDetected and Quantified11183.81 +/- 6864.25 umol/mmol creatinineAdolescent (13-18 years old)Both
Obese
details
UrineDetected and Quantified1.517 umol/mmol creatinineChildren (1-13 years old)Not Available
Carnitine transporter defect; primary systemic carnitine deficiency
details
UrineDetected and Quantified9.395-121.200 umol/mmol creatinineAdult (>18 years old)Female
Myopathic carnitine deficiency
details
UrineDetected and Quantified7.21 umol/mmol creatinineAdult (>18 years old)Not Specified
Propionic acidemia
details
UrineDetected and Quantified7.0 (0.0-30.0) umol/mmol creatinineNot SpecifiedBothLung cancer details
UrineDetected and Quantified17.565 +/- 24.221 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified31-Jul umol/mmol creatinineChildren (1-13 years old)Both
L-2-hydroxyglutaric aciduria
details
Associated Disorders and Diseases
Disease References
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Leung AA, Chan AK, Ezekowitz JA, Leung AK: A Case of Dilated Cardiomyopathy Associated with 3-Hydroxy-3-Methylglutaryl-Coenzyme A (HMG CoA) Lyase Deficiency. Case Rep Med. 2009;2009:183125. doi: 10.1155/2009/183125. Epub 2009 Nov 4. [PubMed:19893767 ]
  2. Dos Santos Mello M, Ribas GS, Wayhs CA, Hammerschmidt T, Guerreiro GB, Favenzani JL, Sitta A, de Moura Coelho D, Wajner M, Vargas CR: Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria. Mol Cell Biochem. 2015 Apr;402(1-2):149-55. doi: 10.1007/s11010-014-2322-x. Epub 2015 Jan 4. [PubMed:25557019 ]
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
  2. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
  3. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ]
  4. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ]
  5. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Perillyl alcohol administration for cancer treatment
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Carnitine palmitoyltransferase I deficiency
  1. Olpin SE, Allen J, Bonham JR, Clark S, Clayton PT, Calvin J, Downing M, Ives K, Jones S, Manning NJ, Pollitt RJ, Standing SJ, Tanner MS: Features of carnitine palmitoyltransferase type I deficiency. J Inherit Metab Dis. 2001 Feb;24(1):35-42. [PubMed:11286380 ]
Colorectal cancer
  1. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ]
  2. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  3. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  4. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Diabetes mellitus type 2
  1. Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. [PubMed:6810706 ]
L-2-Hydroxyglutaric aciduria
  1. Barbot C, Fineza I, Diogo L, Maia M, Melo J, Guimaraes A, Pires MM, Cardoso ML, Vilarinho L: L-2-Hydroxyglutaric aciduria: clinical, biochemical and magnetic resonance imaging in six Portuguese pediatric patients. Brain Dev. 1997 Jun;19(4):268-73. [PubMed:9187477 ]
Lung Cancer
  1. Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 Jan;37(Database issue):D603-10. doi: 10.1093/nar/gkn810. Epub 2008 Oct 25. [PubMed:18953024 ]
Obesity
  1. Cho K, Moon JS, Kang JH, Jang HB, Lee HJ, Park SI, Yu KS, Cho JY: Combined untargeted and targeted metabolomic profiling reveals urinary biomarkers for discriminating obese from normal-weight adolescents. Pediatr Obes. 2017 Apr;12(2):93-101. doi: 10.1111/ijpo.12114. Epub 2016 Feb 22. [PubMed:26910390 ]
  2. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Oculocerebrorenal syndrome
  1. Charnas LR, Bernardini I, Rader D, Hoeg JM, Gahl WA: Clinical and laboratory findings in the oculocerebrorenal syndrome of Lowe, with special reference to growth and renal function. N Engl J Med. 1991 May 9;324(19):1318-25. doi: 10.1056/NEJM199105093241904. [PubMed:2017228 ]
Periodontal disease
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Propionic acidemia
  1. Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
Pancreatic cancer
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
2,4-dienoyl-CoA reductase deficiency
  1. Houten SM, Denis S, Te Brinke H, Jongejan A, van Kampen AH, Bradley EJ, Baas F, Hennekam RC, Millington DS, Young SP, Frazier DM, Gucsavas-Calikoglu M, Wanders RJ: Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia. Hum Mol Genet. 2014 Sep 15;23(18):5009-16. doi: 10.1093/hmg/ddu218. Epub 2014 May 8. [PubMed:24847004 ]
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
  1. Morris AA, Lascelles CV, Olpin SE, Lake BD, Leonard JV, Quant PA: Hepatic mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme a synthase deficiency. Pediatr Res. 1998 Sep;44(3):392-6. doi: 10.1203/00006450-199809000-00021. [PubMed:9727719 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
  2. Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
Carnitine transporter defect; primary systemic carnitine deficiency
  1. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PM: Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts. N Engl J Med. 1988 Nov 17;319(20):1331-6. doi: 10.1056/NEJM198811173192006. [PubMed:3185635 ]
  2. Waber LJ, Valle D, Neill C, DiMauro S, Shug A: Carnitine deficiency presenting as familial cardiomyopathy: a treatable defect in carnitine transport. J Pediatr. 1982 Nov;101(5):700-5. [PubMed:7131143 ]
Long-chain Fatty Acids, Defect in Transport of
  1. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PM: Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts. N Engl J Med. 1988 Nov 17;319(20):1331-6. doi: 10.1056/NEJM198811173192006. [PubMed:3185635 ]
Methylmalonic aciduria mitochondrial encephelopathy Leigh-like
  1. Carrozzo R, Dionisi-Vici C, Steuerwald U, Lucioli S, Deodato F, Di Giandomenico S, Bertini E, Franke B, Kluijtmans LA, Meschini MC, Rizzo C, Piemonte F, Rodenburg R, Santer R, Santorelli FM, van Rooij A, Vermunt-de Koning D, Morava E, Wevers RA: SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness. Brain. 2007 Mar;130(Pt 3):862-74. Epub 2007 Feb 14. [PubMed:17301081 ]
Mitochondrial trifunctional protein deficiency
  1. den Boer ME, Dionisi-Vici C, Chakrapani A, van Thuijl AO, Wanders RJ, Wijburg FA: Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement. J Pediatr. 2003 Jun;142(6):684-9. doi: 10.1067/mpd.2003.231. [PubMed:12838198 ]
Myopathic carnitine deficiency
  1. Vielhaber S, Feistner H, Weis J, Kreuder J, Sailer M, Schroder JM, Kunz WS: Primary carnitine deficiency: adult onset lipid storage myopathy with a mild clinical course. J Clin Neurosci. 2004 Nov;11(8):919-24. doi: 10.1016/j.jocn.2003.11.019. [PubMed:15519880 ]
Early preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Late-onset preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Associated OMIM IDs
  • 114500 (Colorectal cancer)
  • 236792 (L-2-Hydroxyglutaric aciduria)
  • 605911 (3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency)
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 616034 (2,4-dienoyl-CoA reductase deficiency)
  • 212160 (Myopathic carnitine deficiency)
  • 255120 (Carnitine palmitoyltransferase I deficiency)
  • 212140 (Carnitine transporter defect; primary systemic carnitine deficiency)
  • 609015 (Mitochondrial trifunctional protein deficiency)
  • 603376 (Long-chain Fatty Acids, Defect in Transport of)
  • 309000 (Oculocerebrorenal syndrome)
  • 170650 (Periodontal disease)
  • 260350 (Pancreatic cancer)
  • 125853 (Diabetes mellitus type 2)
  • 211980 (Lung Cancer)
  • 610247 (Eosinophilic esophagitis)
  • 606054 (Propionic acidemia)
  • 601665 (Obesity)
DrugBank IDDB00583
Phenol Explorer Compound IDNot Available
FoodDB IDFDB000572
KNApSAcK IDNot Available
Chemspider ID2006614
KEGG Compound IDC00318
BioCyc IDCARNITINE
BiGG ID34600
Wikipedia LinkCarnitine
METLIN ID52
PubChem Compound2724480
PDB ID1NDF
ChEBI ID16347
References
Synthesis Reference Bols, Mikael; Lundt, Inge; Pedersen, Christian. Simple synthesis of (R)-carnitine from D-galactono-1,4-lactone. Tetrahedron (1992), 48(2), 319-24.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Tamai I, China K, Sai Y, Kobayashi D, Nezu J, Kawahara E, Tsuji A: Na(+)-coupled transport of L-carnitine via high-affinity carnitine transporter OCTN2 and its subcellular localization in kidney. Biochim Biophys Acta. 2001 Jun 6;1512(2):273-84. [PubMed:11406104 ]
  2. Waldner R, Laschan C, Lohninger A, Gessner M, Tuchler H, Huemer M, Spiegel W, Karlic H: Effects of doxorubicin-containing chemotherapy and a combination with L-carnitine on oxidative metabolism in patients with non-Hodgkin lymphoma. J Cancer Res Clin Oncol. 2006 Feb;132(2):121-8. Epub 2005 Nov 8. [PubMed:16283381 ]
  3. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  4. Wachter S, Vogt M, Kreis R, Boesch C, Bigler P, Hoppeler H, Krahenbuhl S: Long-term administration of L-carnitine to humans: effect on skeletal muscle carnitine content and physical performance. Clin Chim Acta. 2002 Apr;318(1-2):51-61. [PubMed:11880112 ]
  5. Evans AM, Fornasini G: Pharmacokinetics of L-carnitine. Clin Pharmacokinet. 2003;42(11):941-67. [PubMed:12908852 ]
  6. Pastoris O, Dossena M, Foppa P, Catapano M, Arbustini E, Bellini O, Dal Bello B, Minzioni G, Ceriana P, Barzaghi N: Effect of L-carnitine on myocardial metabolism: results of a balanced, placebo-controlled, double-blind study in patients undergoing open heart surgery. Pharmacol Res. 1998 Feb;37(2):115-22. [PubMed:9572066 ]
  7. Stephens FB, Constantin-Teodosiu D, Laithwaite D, Simpson EJ, Greenhaff PL: Insulin stimulates L-carnitine accumulation in human skeletal muscle. FASEB J. 2006 Feb;20(2):377-9. Epub 2005 Dec 20. [PubMed:16368715 ]
  8. Malaguarnera M, Pistone G, Astuto M, Dell'Arte S, Finocchiaro G, Lo Giudice E, Pennisi G: L-Carnitine in the treatment of mild or moderate hepatic encephalopathy. Dig Dis. 2003;21(3):271-5. [PubMed:14571103 ]
  9. Oey NA, van Vlies N, Wijburg FA, Wanders RJ, Attie-Bitach T, Vaz FM: L-carnitine is synthesized in the human fetal-placental unit: potential roles in placental and fetal metabolism. Placenta. 2006 Aug;27(8):841-6. Epub 2005 Nov 18. [PubMed:16300828 ]
  10. Feinfeld DA, Kurian P, Cheng JT, Dilimetin G, Arriola MR, Ward L, Manis T, Carvounis CP: Effect of oral L-carnitine on serum myoglobin in hemodialysis patients. Ren Fail. 1996 Jan;18(1):91-6. [PubMed:8820505 ]
  11. Matalliotakis I, Koumantaki Y, Evageliou A, Matalliotakis G, Goumenou A, Koumantakis E: L-carnitine levels in the seminal plasma of fertile and infertile men: correlation with sperm quality. Int J Fertil Womens Med. 2000 May-Jun;45(3):236-40. [PubMed:10929687 ]
  12. Vescovo G, Ravara B, Gobbo V, Dalla Libera L: Inflammation and perturbation of the l-carnitine system in heart failure. Eur J Heart Fail. 2005 Oct;7(6):997-1002. [PubMed:16227137 ]
  13. Lerch R: [The effect of L-carnitine on ischemic heart disease: experimental results]. Praxis (Bern 1994). 1998 Jan 21;87(4):97-100. [PubMed:9522638 ]
  14. Khademi A, Alleyassin A, Safdarian L, Hamed EA, Rabiee E, Haghaninezhad H: The effects of L-carnitine on sperm parameters in smoker and non-smoker patients with idiopathic sperm abnormalities. J Assist Reprod Genet. 2005 Dec;22(11-12):395-9. [PubMed:16331536 ]
  15. Stradomska TJ, Tylki-Szymanska A, Bentkowski Z: Very long-chain fatty acids in Rett syndrome. Eur J Pediatr. 1999 Mar;158(3):226-9. [PubMed:10094444 ]
  16. Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. [PubMed:6810706 ]
  17. Lenzi A, Sgro P, Salacone P, Paoli D, Gilio B, Lombardo F, Santulli M, Agarwal A, Gandini L: A placebo-controlled double-blind randomized trial of the use of combined l-carnitine and l-acetyl-carnitine treatment in men with asthenozoospermia. Fertil Steril. 2004 Jun;81(6):1578-84. [PubMed:15193480 ]
  18. Sinclair C, Gilchrist JM, Hennessey JV, Kandula M: Muscle carnitine in hypo- and hyperthyroidism. Muscle Nerve. 2005 Sep;32(3):357-9. [PubMed:15803480 ]
  19. Ahmad S: L-carnitine in dialysis patients. Semin Dial. 2001 May-Jun;14(3):209-17. [PubMed:11422928 ]
  20. Shihabi ZK, Oles KS, McCormick CP, Penry JK: Serum and tissue carnitine assay based on dialysis. Clin Chem. 1992 Aug;38(8 Pt 1):1414-7. [PubMed:1643708 ]
  21. Steiber A, Kerner J, Hoppel CL: Carnitine: a nutritional, biosynthetic, and functional perspective. Mol Aspects Med. 2004 Oct-Dec;25(5-6):455-73. [PubMed:15363636 ]
  22. Olpin SE: Fatty acid oxidation defects as a cause of neuromyopathic disease in infants and adults. Clin Lab. 2005;51(5-6):289-306. [PubMed:15991803 ]
  23. Borum PR, York CM, Broquist HP: Carnitine content of liquid formulas and special diets. Am J Clin Nutr. 1979 Nov;32(11):2272-6. doi: 10.1093/ajcn/32.11.2272. [PubMed:115309 ]

Only showing the first 10 proteins. There are 16 proteins in total.

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
Reactions
Acetyl-CoA + L-Carnitine → Coenzyme A + L-Acetylcarnitinedetails
References
  1. Jogl G, Tong L: Crystal structure of carnitine acetyltransferase and implications for the catalytic mechanism and fatty acid transport. Cell. 2003 Jan 10;112(1):113-22. [PubMed:12526798 ]
  2. Wu D, Govindasamy L, Lian W, Gu Y, Kukar T, Agbandje-McKenna M, McKenna R: Structure of human carnitine acetyltransferase. Molecular basis for fatty acyl transfer. J Biol Chem. 2003 Apr 11;278(15):13159-65. Epub 2003 Jan 31. [PubMed:12562770 ]
  3. Vikramadithyan RK, Hiriyan J, Suresh J, Gershome C, Babu RK, Misra P, Rajagopalan R, Chakrabarti R: DRF 2655: a unique molecule that reduces body weight and ameliorates metabolic abnormalities. Obes Res. 2003 Feb;11(2):292-303. [PubMed:12582227 ]
  4. Govindasamy L, Kukar T, Lian W, Pedersen B, Gu Y, Agbandje-McKenna M, Jin S, McKenna R, Wu D: Structural and mutational characterization of L-carnitine binding to human carnitine acetyltransferase. J Struct Biol. 2004 Jun;146(3):416-24. [PubMed:15099582 ]
  5. Cordente AG, Lopez-Vinas E, Vazquez MI, Swiegers JH, Pretorius IS, Gomez-Puertas P, Hegardt FG, Asins G, Serra D: Redesign of carnitine acetyltransferase specificity by protein engineering. J Biol Chem. 2004 Aug 6;279(32):33899-908. Epub 2004 May 21. [PubMed:15155769 ]
General function:
Involved in oxidoreductase activity
Specific function:
Key enzyme in purine degradation. Catalyzes the oxidation of hypoxanthine to xanthine. Catalyzes the oxidation of xanthine to uric acid. Contributes to the generation of reactive oxygen species. Has also low oxidase activity towards aldehydes (in vitro).
Gene Name:
XDH
Uniprot ID:
P47989
Molecular weight:
146422.99
References
  1. Di Giacomo C, Latteri F, Fichera C, Sorrenti V, Campisi A, Castorina C, Russo A, Pinturo R, Vanella A: Effect of acetyl-L-carnitine on lipid peroxidation and xanthine oxidase activity in rat skeletal muscle. Neurochem Res. 1993 Nov;18(11):1157-62. [PubMed:8255367 ]
General function:
Involved in peroxidase activity
Specific function:
Part of the host defense system of polymorphonuclear leukocytes. It is responsible for microbicidal activity against a wide range of organisms. In the stimulated PMN, MPO catalyzes the production of hypohalous acids, primarily hypochlorous acid in physiologic situations, and other toxic intermediates that greatly enhance PMN microbicidal activity.
Gene Name:
MPO
Uniprot ID:
P05164
Molecular weight:
83867.71
References
  1. Derin N, Agac A, Bayram Z, Asar M, Izgut-Uysal VN: Effects of L-carnitine on neutrophil-mediated ischemia-reperfusion injury in rat stomach. Cell Biochem Funct. 2006 Sep-Oct;24(5):437-42. [PubMed:16130180 ]
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
Reactions
Octanoyl-CoA + L-Carnitine → Coenzyme A + L-Octanoylcarnitinedetails
References
  1. Cordente AG, Lopez-Vinas E, Vazquez MI, Swiegers JH, Pretorius IS, Gomez-Puertas P, Hegardt FG, Asins G, Serra D: Redesign of carnitine acetyltransferase specificity by protein engineering. J Biol Chem. 2004 Aug 6;279(32):33899-908. Epub 2004 May 21. [PubMed:15155769 ]
  2. Cordente AG, Lopez-Vinas E, Vazquez MI, Gomez-Puertas P, Asins G, Serra D, Hegardt FG: Mutagenesis of specific amino acids converts carnitine acetyltransferase into carnitine palmitoyltransferase. Biochemistry. 2006 May 16;45(19):6133-41. [PubMed:16681386 ]
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT1B
Uniprot ID:
Q92523
Molecular weight:
83890.705
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-Palmitoylcarnitinedetails
General function:
Involved in acyltransferase activity
Specific function:
Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism.
Gene Name:
CPT1A
Uniprot ID:
P50416
Molecular weight:
86238.415
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-Palmitoylcarnitinedetails
References
  1. Xu ZR, Wang MQ, Mao HX, Zhan XA, Hu CH: Effects of L-carnitine on growth performance, carcass composition, and metabolism of lipids in male broilers. Poult Sci. 2003 Mar;82(3):408-13. [PubMed:12705401 ]
  2. Morillas M, Lopez-Vinas E, Valencia A, Serra D, Gomez-Puertas P, Hegardt FG, Asins G: Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal. Biochem J. 2004 May 1;379(Pt 3):777-84. [PubMed:14711372 ]
  3. Tripodi G, Modica R, Stella A, Bigatti G, Bianchi G, Stella P: Haplotype analysis of carnitine transporters and left ventricular mass in human essential hypertension. J Ren Nutr. 2005 Jan;15(1):2-7. [PubMed:15647998 ]
  4. Waldner R, Laschan C, Lohninger A, Gessner M, Tuchler H, Huemer M, Spiegel W, Karlic H: Effects of doxorubicin-containing chemotherapy and a combination with L-carnitine on oxidative metabolism in patients with non-Hodgkin lymphoma. J Cancer Res Clin Oncol. 2006 Feb;132(2):121-8. Epub 2005 Nov 8. [PubMed:16283381 ]
  5. Shin ES, Cho SY, Lee EH, Lee SJ, Chang IS, Lee TR: Positive regulation of hepatic carnitine palmitoyl transferase 1A (CPT1A) activities by soy isoflavones and L-carnitine. Eur J Nutr. 2006 Mar;45(3):159-64. Epub 2005 Dec 20. [PubMed:16362726 ]
  6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT2
Uniprot ID:
P23786
Molecular weight:
73776.335
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-Palmitoylcarnitinedetails
References
  1. Barrero MJ, Camarero N, Marrero PF, Haro D: Control of human carnitine palmitoyltransferase II gene transcription by peroxisome proliferator-activated receptor through a partially conserved peroxisome proliferator-responsive element. Biochem J. 2003 Feb 1;369(Pt 3):721-9. [PubMed:12408750 ]
  2. Kong JY, Rabkin SW: Lovastatin does not accentuate but is rather additive to palmitate-induced apoptosis in cardiomyocytes. Prostaglandins Leukot Essent Fatty Acids. 2002 Nov;67(5):293-302. [PubMed:12445488 ]
  3. Rasmussen BB, Holmback UC, Volpi E, Morio-Liondore B, Paddon-Jones D, Wolfe RR: Malonyl coenzyme A and the regulation of functional carnitine palmitoyltransferase-1 activity and fat oxidation in human skeletal muscle. J Clin Invest. 2002 Dec;110(11):1687-93. [PubMed:12464674 ]
  4. Price NT, Jackson VN, van der Leij FR, Cameron JM, Travers MT, Bartelds B, Huijkman NC, Zammit VA: Cloning and expression of the liver and muscle isoforms of ovine carnitine palmitoyltransferase 1: residues within the N-terminus of the muscle isoform influence the kinetic properties of the enzyme. Biochem J. 2003 Jun 15;372(Pt 3):871-9. [PubMed:12662154 ]
  5. Lehtihet M, Welsh N, Berggren PO, Cook GA, Sjoholm A: Glibenclamide inhibits islet carnitine palmitoyltransferase 1 activity, leading to PKC-dependent insulin exocytosis. Am J Physiol Endocrinol Metab. 2003 Aug;285(2):E438-46. Epub 2003 Apr 8. [PubMed:12684219 ]
General function:
Involved in acyltransferase activity
Specific function:
Not Available
Gene Name:
CPT1C
Uniprot ID:
Q8TCG5
Molecular weight:
89712.575
Reactions
hexadecanoyl-CoA + L-Carnitine → Coenzyme A + L-Palmitoylcarnitinedetails
General function:
Involved in iron ion binding
Specific function:
Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
Gene Name:
BBOX1
Uniprot ID:
O75936
Molecular weight:
44714.6
Reactions
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + CO(2)details
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + Carbon dioxidedetails
General function:
Lipid transport and metabolism
Specific function:
Involved in the detoxification of xenobiotics and in the activation of ester and amide prodrugs. Hydrolyzes aromatic and aliphatic esters, but has no catalytic activity toward amides or a fatty acyl-CoA ester. Hydrolyzes the methyl ester group of cocaine to form benzoylecgonine. Catalyzes the transesterification of cocaine to form cocaethylene. Displays fatty acid ethyl ester synthase activity, catalyzing the ethyl esterification of oleic acid to ethyloleate.
Gene Name:
CES1
Uniprot ID:
P23141
Molecular weight:
62520.62
References
  1. Bell FP: Carnitine ester hydrolysis in arteries from normal and cholesterol-fed rabbits and the effects of carnitine esters on arterial microsomal ACAT. Comp Biochem Physiol B. 1984;79(2):125-8. [PubMed:6509906 ]

Transporters

General function:
Involved in transporter activity
Specific function:
Mediates the Na(+)-independent transport of organic anions such as pravastatin, taurocholate, methotrexate, dehydroepiandrosterone sulfate, 17-beta-glucuronosyl estradiol, estrone sulfate, prostaglandin E2, thromboxane B2, leukotriene C3, leukotriene E4, thyroxine and triiodothyronine. May play an important role in the clearance of bile acids and organic anions from the liver
Gene Name:
SLCO1B1
Uniprot ID:
Q9Y6L6
Molecular weight:
76448.0
References
  1. Nozawa T, Tamai I, Sai Y, Nezu J, Tsuji A: Contribution of organic anion transporting polypeptide OATP-C to hepatic elimination of the opioid pentapeptide analogue [D-Ala2, D-Leu5]-enkephalin. J Pharm Pharmacol. 2003 Jul;55(7):1013-20. [PubMed:12906759 ]
General function:
Involved in ion transmembrane transporter activity
Specific function:
Sodium-ion dependent, high affinity carnitine transporter. Involved in the active cellular uptake of carnitine. Transports one sodium ion with one molecule of carnitine. Also transports organic cations such as tetraethylammonium (TEA) without the involvement of sodium. Also relative uptake activity ratio of carnitine to TEA is 11.3
Gene Name:
SLC22A5
Uniprot ID:
O76082
Molecular weight:
62751.1
References
  1. Lahjouji K, Elimrani I, Wu J, Mitchell GA, Qureshi IA: A heterozygote phenotype is present in the jvs +/- mutant mouse livers. Mol Genet Metab. 2002 May;76(1):76-80. [PubMed:12175785 ]
  2. Kristufek D, Rudorfer W, Pifl C, Huck S: Organic cation transporter mRNA and function in the rat superior cervical ganglion. J Physiol. 2002 Aug 15;543(Pt 1):117-34. [PubMed:12181285 ]
  3. Ohashi R, Tamai I, Inano A, Katsura M, Sai Y, Nezu J, Tsuji A: Studies on functional sites of organic cation/carnitine transporter OCTN2 (SLC22A5) using a Ser467Cys mutant protein. J Pharmacol Exp Ther. 2002 Sep;302(3):1286-94. [PubMed:12183691 ]
  4. Hou JW: Primary systemic carnitine deficiency presenting as recurrent Reye-like syndrome and dilated cardiomyopathy. Chang Gung Med J. 2002 Dec;25(12):832-7. [PubMed:12635840 ]
  5. Friedrich A, Prasad PD, Freyer D, Ganapathy V, Brust P: Molecular cloning and functional characterization of the OCTN2 transporter at the RBE4 cells, an in vitro model of the blood-brain barrier. Brain Res. 2003 Apr 4;968(1):69-79. [PubMed:12644265 ]
  6. Tamai I, China K, Sai Y, Kobayashi D, Nezu J, Kawahara E, Tsuji A: Na(+)-coupled transport of L-carnitine via high-affinity carnitine transporter OCTN2 and its subcellular localization in kidney. Biochim Biophys Acta. 2001 Jun 6;1512(2):273-84. [PubMed:11406104 ]
  7. Elimrani I, Lahjouji K, Seidman E, Roy MJ, Mitchell GA, Qureshi I: Expression and localization of organic cation/carnitine transporter OCTN2 in Caco-2 cells. Am J Physiol Gastrointest Liver Physiol. 2003 May;284(5):G863-71. Epub 2003 Jan 10. [PubMed:12684216 ]
  8. Ohashi R, Tamai I, Yabuuchi H, Nezu JI, Oku A, Sai Y, Shimane M, Tsuji A: Na(+)-dependent carnitine transport by organic cation transporter (OCTN2): its pharmacological and toxicological relevance. J Pharmacol Exp Ther. 1999 Nov;291(2):778-84. [PubMed:10525100 ]
  9. Tamai I, Ohashi R, Nezu JI, Sai Y, Kobayashi D, Oku A, Shimane M, Tsuji A: Molecular and functional characterization of organic cation/carnitine transporter family in mice. J Biol Chem. 2000 Dec 22;275(51):40064-72. [PubMed:11010964 ]
  10. Wu X, Huang W, Prasad PD, Seth P, Rajan DP, Leibach FH, Chen J, Conway SJ, Ganapathy V: Functional characteristics and tissue distribution pattern of organic cation transporter 2 (OCTN2), an organic cation/carnitine transporter. J Pharmacol Exp Ther. 1999 Sep;290(3):1482-92. [PubMed:10454528 ]
General function:
Involved in ion transmembrane transporter activity
Specific function:
Sodium-ion dependent, low affinity carnitine transporter. Probably transports one sodium ion with one molecule of carnitine. Also transports organic cations such as tetraethylammonium (TEA) without the involvement of sodium. Relative uptake activity ratio of carnitine to TEA is 1.78. A key substrate of this transporter seems to be ergothioneine (ET)
Gene Name:
SLC22A4
Uniprot ID:
Q9H015
Molecular weight:
62154.5
References
  1. Kristufek D, Rudorfer W, Pifl C, Huck S: Organic cation transporter mRNA and function in the rat superior cervical ganglion. J Physiol. 2002 Aug 15;543(Pt 1):117-34. [PubMed:12181285 ]
  2. Amat di San Filippo C, Wang Y, Longo N: Functional domains in the carnitine transporter OCTN2, defective in primary carnitine deficiency. J Biol Chem. 2003 Nov 28;278(48):47776-84. Epub 2003 Sep 23. [PubMed:14506273 ]
  3. Lamhonwah AM, Ackerley C, Onizuka R, Tilups A, Lamhonwah D, Chung C, Tao KS, Tellier R, Tein I: Epitope shared by functional variant of organic cation/carnitine transporter, OCTN1, Campylobacter jejuni and Mycobacterium paratuberculosis may underlie susceptibility to Crohn's disease at 5q31. Biochem Biophys Res Commun. 2005 Dec 2;337(4):1165-75. Epub 2005 Oct 6. [PubMed:16246312 ]
  4. Lash LH, Putt DA, Cai H: Membrane transport function in primary cultures of human proximal tubular cells. Toxicology. 2006 Dec 7;228(2-3):200-18. Epub 2006 Sep 1. [PubMed:16997449 ]
  5. Yabuuchi H, Tamai I, Nezu J, Sakamoto K, Oku A, Shimane M, Sai Y, Tsuji A: Novel membrane transporter OCTN1 mediates multispecific, bidirectional, and pH-dependent transport of organic cations. J Pharmacol Exp Ther. 1999 May;289(2):768-73. [PubMed:10215651 ]
  6. Tamai I, Ohashi R, Nezu JI, Sai Y, Kobayashi D, Oku A, Shimane M, Tsuji A: Molecular and functional characterization of organic cation/carnitine transporter family in mice. J Biol Chem. 2000 Dec 22;275(51):40064-72. [PubMed:11010964 ]
General function:
Involved in transporter activity
Specific function:
High affinity carnitine transporter; the uptake is partially sodium-ion dependent. Thought to mediate the L-carnitine secretion mechanism from testis epididymal epithelium into the lumen which is involved in the maturation of spermatozoa. Also transports organic cations such as tetraethylammonium (TEA) and doxorubicin. The uptake of TEA is inhibited by various organic cations. The uptake of doxorubicin is sodium-independent
Gene Name:
SLC22A16
Uniprot ID:
Q86VW1
Molecular weight:
64613.6
References
  1. Enomoto A, Wempe MF, Tsuchida H, Shin HJ, Cha SH, Anzai N, Goto A, Sakamoto A, Niwa T, Kanai Y, Anders MW, Endou H: Molecular identification of a novel carnitine transporter specific to human testis. Insights into the mechanism of carnitine recognition. J Biol Chem. 2002 Sep 27;277(39):36262-71. Epub 2002 Jun 27. [PubMed:12089149 ]

Only showing the first 10 proteins. There are 16 proteins in total.