| Record Information |
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| Version | 2.0 |
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| Creation Date | 2009-07-21 20:27:12 UTC |
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| Update Date | 2014-12-24 20:25:52 UTC |
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| Accession Number | T3D2832 |
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| Identification |
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| Common Name | L-Carnitine |
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| Class | Small Molecule |
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| Description | Carnitine 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 most usually employed by the body in the construction of protein. Carnitine is an essential factor in fatty acid metabolism in mammals. It's 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. Borurn et al. (1979) describe carnitine as an essential nutrient for pre-term babies, certain types (non-ketotic) of hypoglycemics, kidney dialysis patients, cirrhosis, and in kwashiorkor, type IV hyperlipidemia, heart muscle disease (cardiomyopathy), and propionic or organic aciduria (acid urine resulting from genetic or other anomalies). In all these conditions and the inborn errors of carnitine metabolism, 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 be worth a trial in any form of hyperlipidemia or muscle weakness. carnitine supplements may 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 low motility of sperm. Even the Physician's Desk Reference gives indication for carnitine supplements as improving the tolerance of ischemic heart disease, myocardial insufficiencies, and type IV hyperlipoproteinemia. carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia. |
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| Compound Type | - Dietary Supplement
- Drug
- Food Toxin
- Metabolite
- Natural Compound
- Nootropic Agent
- Nutraceutical
- Organic Compound
- Vitamin B Complex
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| Chemical Structure | |
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| Synonyms | | Synonym | | (-)-(R)-3-Hydroxy-4-(trimethylammonio)butyrate | | (-)-carnitine | | (-)-L-Carnitin | | (-)-L-Carnitine | | (R)-(3-Carboxy-2-hydroxypropyl)trimethylammonium hydroxide | | (R)-carnitine | | (S)-carnitine | | 1-Carnitine | | 3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium | | 3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium hydroxide, inner salt | | 3-Hydroxy-4-trimethylammoniobutanoate | | 3-Hydroxy-4-trimethylammoniobutanoic acid | | Albicar | | Aveptol | | Bicarnesine | | Biocarn | | Bitobionil | | Cardimax | | Cardispan | | Carniben | | Carnicor | | Carniking | | Carniking 50 | | Carnilean | | Carnipass | | Carnipass 20 | | Carnitene | | Carnitine | | Carnitor | | D-Carnitine | | delta-Carnitine | | DL-carnitine | | gamma-Trimethyl-ammonium-beta-hydroxybutirate | | gamma-Trimethyl-beta-hydroxybutyrobetaine | | gamma-Trimethyl-hydroxybutyrobetaine | | Karnitin | | L-(-)-Carnitine | | L-gamma-Trimethyl-beta-hydroxybutyrobetaine | | Lecarna | | Levocarnitin | | Levocarnitina | | Levocarnitine | | Levocarnitinum | | R-(-)-3-Hydroxy-4-trimethylaminobutyrate | | Vitamin BT |
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| Chemical Formula | C7H15NO3 |
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| Average Molecular Mass | 161.199 g/mol |
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| Monoisotopic Mass | 161.105 g/mol |
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| CAS Registry Number | 541-15-1 |
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| IUPAC Name | (3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate |
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| Traditional Name | L-carnitine |
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| SMILES | [H][C@@](O)(CC([O-])=O)C[N+](C)(C)C |
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| 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 |
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| InChI Key | InChIKey=PHIQHXFUZVPYII-ZCFIWIBFSA-N |
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| Chemical Taxonomy |
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| Description | belongs to the class of organic compounds known as carnitines. These are organic compounds containing the quaternary ammonium compound carnitine. |
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| Kingdom | Organic compounds |
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| Super Class | Organic nitrogen compounds |
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| Class | Organonitrogen compounds |
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| Sub Class | Quaternary ammonium salts |
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| Direct Parent | Carnitines |
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| Alternative Parents | |
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| 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
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| Molecular Framework | Aliphatic acyclic compounds |
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| External Descriptors | |
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| Biological Properties |
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| Status | Detected and Not Quantified |
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| Origin | Endogenous |
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| Cellular Locations | - Cytoplasm
- Endoplasmic reticulum
- Extracellular
- Membrane
- Mitochondria
- Peroxisome
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| Biofluid Locations | Not Available |
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| Tissue Locations | - Adipose Tissue
- Bladder
- Brain
- Erythrocyte
- Fibroblasts
- Intestine
- Kidney
- Liver
- Lung
- Muscle
- Myocardium
- Nerve Cells
- Neuron
- Platelet
- Prostate
- Skeletal Muscle
- Sperm
- Testes
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| Pathways | | Name | SMPDB Link | KEGG Link |
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| Beta Oxidation of Very Long Chain Fatty Acids | SMP00052 | map01040 | | Carnitine Synthesis | SMP00465 | Not Available | | Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids | SMP00482 | Not Available | | Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids | SMP00480 | Not Available | | Oxidation of Branched Chain Fatty Acids | SMP00030 | Not Available | | Carnitine palmitoyl transferase deficiency (I) | SMP00538 | Not Available | | Lysinuric Protein Intolerance | SMP00197 | Not Available | | Propionic Acidemia | SMP00236 | Not Available |
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| Applications | |
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| Biological Roles | |
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| Chemical Roles | Not Available |
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| Physical Properties |
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| State | Solid |
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| Appearance | White powder. |
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| Experimental Properties | | Property | Value |
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| Melting Point | 195-198°C | | Boiling Point | Not Available | | Solubility | 2500 mg/mL | | LogP | Not Available |
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| Predicted Properties | |
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| Spectra |
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| Spectra | | Spectrum Type | Description | Splash Key | Deposition Date | View |
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| Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | splash10-00di-9100000000-1b25dacb04c3ed5be8d0 | 2016-09-22 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive | splash10-00di-9100000000-c15eaa190d1e28a4839a | 2017-10-06 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | Not Available | 2021-10-12 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TMS_2_1) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | | Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive | Not Available | 2021-11-05 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , positive | splash10-03di-0900000000-9b579c570aab7c7d3a21 | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , positive | splash10-0ik9-0900000000-e37d3def2de1af5bd2f5 | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , positive | splash10-0ue9-8900000000-cf37f0a721cd52d86e8e | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , positive | splash10-0r0c-9200000000-a11a4872036f3c33d25a | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QQ , positive | splash10-0a4l-9000000000-2402e260a330ff1772a1 | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-IT , positive | splash10-0w29-6900000000-d0136248acdd706e650b | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QTOF , positive | splash10-0ik9-2900000000-61732bb10307f2b183ca | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QTOF , positive | splash10-0zg0-9400000000-15c3db3324f1d46d3c7c | 2017-09-14 | View Spectrum | | LC-MS/MS | LC-MS/MS Spectrum - LC-ESI-QTOF , positive | splash10-0ik9-2900000000-ee79f10dce9fc09246ec | 2017-09-14 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-01ox-0900000000-3bffd5143cf8b072299e | 2017-09-01 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-00kf-0900000000-e68f553bcb15d3ef5b47 | 2017-09-01 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-00di-9200000000-c5b43723951af6da48cf | 2017-09-01 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-03di-1900000000-dd1b1a023937cdcb11af | 2017-09-01 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-114i-3900000000-9f24d3bc535736149e2a | 2017-09-01 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-0a4l-9000000000-632ef17d9dde38586990 | 2017-09-01 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-03di-3900000000-97af6636a917c0edea61 | 2021-09-24 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-01pc-9300000000-543e21cb6b9785149e31 | 2021-09-24 | View Spectrum | | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-08fr-9000000000-9128d6fe96d9c7276695 | 2021-09-24 | View Spectrum | | 1D NMR | 1H NMR Spectrum (1D, D2O, experimental) | Not Available | 2016-10-22 | View Spectrum |
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| Toxicity Profile |
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| Route of Exposure | Intravenous, Oral.
Absolute bioavailability is 15% (tablets or solution).
Time to maximum plasma concentration was found to be 3.3 hours. |
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| Mechanism of Toxicity | Levocarnitine can be synthesised within the body from the amino acids lysine or methionine. Vitamin C (ascorbic acid) is essential to the synthesis of carnitine. Levocarnitine is a carrier molecule in the transport of long chain fatty acids across the inner mitochondrial membrane. It also exports acyl groups from subcellular organelles and from cells to urine before they accumulate to toxic concentrations. Only the L isomer of carnitine (sometimes called vitamin BT) affects lipid metabolism. Levocarnitine is handled by several proteins in different pathways including carnitine transporters, carnitine translocases, carnitine acetyltransferases and carnitine palmitoyltransferases. |
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| Metabolism | After oral administration L-carnitine which is unabsorbed is metabolized in the gastrointestinal tract by bacterial microflora. Major metabolites include trimethylamine N-oxide and [3H]-gamma-butyrobetaine.
Route of Elimination: Following a single intravenous dose, 73.1 +/- 16% of the dose was excreted in the urine during the 0-24 hour interval.
Post administration of oral carnitine supplements, in addition to a high carnitine diet, 58-65% of the administered radioactive dose was recovered from urine and feces in 5-11 days.
Half Life: 17.4 hours (elimination) following a single intravenous dose. |
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| Toxicity Values | LD50 > 8g/kg (mouse, oral). |
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| Lethal Dose | Not Available |
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| Carcinogenicity (IARC Classification) | No indication of carcinogenicity to humans (not listed by IARC). |
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| Uses/Sources | For treatment of primary systemic carnitine deficiency, a genetic impairment of normal biosynthesis or utilization of levocarnitine from dietary sources, or for the treatment of secondary carnitine deficiency resulting from an inborn error of metabolism such as glutaric aciduria II, methyl malonic aciduria, propionic acidemia, and medium chain fatty acylCoA dehydrogenase deficiency. Used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias. Parenteral levocarnitine is indicated for the prevention and treatment of carnitine deficiency in patients with end-stage renal disease. |
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| Minimum Risk Level | Not Available |
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| Health Effects | Not Available |
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| Symptoms | Adverse effects include hypertension, fever, tachycardia and seizures. |
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| Treatment | Not Available |
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| Normal Concentrations |
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| Not Available |
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| Abnormal Concentrations |
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| Not Available |
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| External Links |
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| DrugBank ID | DB00583 |
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| HMDB ID | HMDB00062 |
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| PubChem Compound ID | 10917 |
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| ChEMBL ID | CHEMBL1149 |
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| ChemSpider ID | 10455 |
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| KEGG ID | C00318 |
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| UniProt ID | Not Available |
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| OMIM ID | 201450 , 212138 , 212140 , 212160 , 236795 , 255120 |
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| ChEBI ID | 16347 |
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| BioCyc ID | CARNITINE |
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| CTD ID | Not Available |
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| Stitch ID | L-Carnitine |
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| PDB ID | 152 |
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| ACToR ID | Not Available |
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| Wikipedia Link | L-Carnitine |
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| References |
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| Synthesis Reference | Noguchi, J. and Sakota, N.; US. Patent 3,135,788; June 2,1964; assigned to Nihon Zoki Seiyaku Kabushikikaisha (Japan). |
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| MSDS | Link |
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| General References | - Olpin SE: Fatty acid oxidation defects as a cause of neuromyopathic disease in infants and adults. Clin Lab. 2005;51(5-6):289-306. [15991803 ]
- Steiber A, Kerner J, Hoppel CL: Carnitine: a nutritional, biosynthetic, and functional perspective. Mol Aspects Med. 2004 Oct-Dec;25(5-6):455-73. [15363636 ]
- 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. [11880112 ]
- Evans AM, Fornasini G: Pharmacokinetics of L-carnitine. Clin Pharmacokinet. 2003;42(11):941-67. [12908852 ]
- 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. [9572066 ]
- 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. [16368715 ]
- 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. [11406104 ]
- 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. [14571103 ]
- 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. [16300828 ]
- 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. [8820505 ]
- 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. [10929687 ]
- 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. [16227137 ]
- Lerch R: [The effect of L-carnitine on ischemic heart disease: experimental results]. Praxis (Bern 1994). 1998 Jan 21;87(4):97-100. [9522638 ]
- 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. [16331536 ]
- Stradomska TJ, Tylki-Szymanska A, Bentkowski Z: Very long-chain fatty acids in Rett syndrome. Eur J Pediatr. 1999 Mar;158(3):226-9. [10094444 ]
- Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. [6810706 ]
- 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. [16283381 ]
- 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. [15193480 ]
- Sinclair C, Gilchrist JM, Hennessey JV, Kandula M: Muscle carnitine in hypo- and hyperthyroidism. Muscle Nerve. 2005 Sep;32(3):357-9. [15803480 ]
- Ahmad S: L-carnitine in dialysis patients. Semin Dial. 2001 May-Jun;14(3):209-17. [11422928 ]
- 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. [1643708 ]
- 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. [19212411 ]
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| Gene Regulation |
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| Up-Regulated Genes | Not Available |
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| Down-Regulated Genes | Not Available |
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