L-Carnitine
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Basic Info
Common Name | L-Carnitine(F04760) |
2D Structure | |
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. |
FRCD ID | F04760 |
CAS Number | 541-15-1 |
PubChem CID | 10917 |
Formula | C7H15NO3 |
IUPAC Name | (3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate |
InChI Key | PHIQHXFUZVPYII-ZCFIWIBFSA-N |
InChI | InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1 |
Canonical SMILES | C[N+](C)(C)CC(CC(=O)[O-])O |
Isomeric SMILES | C[N+](C)(C)C[C@@H](CC(=O)[O-])O |
Wikipedia | L-Carnitine |
Synonyms | (-)-L-Carnitine L-carnitine Levocarnitine 541-15-1 vitamin BT (R)-Carnitine Carnitor (-)-Carnitine Carnitine Karnitin |
Classifies | Predicted: Animal Toxin |
Update Date | Nov 13, 2018 17:07 |
Chemical Taxonomy
Kingdom | Organic compounds |
Superclass | Organic nitrogen compounds |
Class | Organonitrogen compounds |
Subclass | Quaternary ammonium salts |
Intermediate Tree Nodes | Not available |
Direct Parent | Carnitines |
Alternative Parents |
|
Molecular Framework | Aliphatic acyclic compounds |
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 |
Description | This compound belongs to the class of organic compounds known as carnitines. These are organic compounds containing the quaternary ammonium compound carnitine. |
Properties
Property Name | Property Value |
---|---|
Molecular Weight | 161.201 |
Hydrogen Bond Donor Count | 1 |
Hydrogen Bond Acceptor Count | 3 |
Rotatable Bond Count | 3 |
Complexity | 134 |
Monoisotopic Mass | 161.105 |
Exact Mass | 161.105 |
XLogP | -0.2 |
Formal Charge | 0 |
Heavy Atom Count | 11 |
Defined Atom Stereocenter Count | 1 |
Undefined Atom Stereocenter Count | 0 |
Defined Bond Stereocenter Count | 0 |
Undefined Bond Stereocenter Count | 0 |
Isotope Atom Count | 0 |
Covalently-Bonded Unit Count | 1 |
References
Title | Journal | Date | Pubmed ID |
---|---|---|---|
The excessive use of antioxidant therapy: A possible cause of male infertility? | Andrologia | 2018 Sep 26 | 30259539 |
Effect of two treatment protocols for ketosis on the resolution, postpartumhealth, milk yield, and reproductive outcomes of dairy cows. | Theriogenology | 2018 Jan 15 | 29035838 |
Metabonomic analysis of toxic action of long-term low-level exposure to acrylamide in rat serum. | Hum Exp Toxicol | 2018 Jan 1 | 29658313 |
Metabolomic analysis of the toxic effect of chronic exposure of cadmium on rat urine. | Environ Sci Pollut Res Int | 2018 Feb | 29168138 |
Development of High Capacity Enterosorbents for Aflatoxin B1 and Other Hazardous Chemicals. | Chem Res Toxicol | 2017 Sep 18 | 28768106 |
Inhibited fatty acid β-oxidation impairs stress resistance ability in Niletilapia (Oreochromis niloticus). | Fish Shellfish Immunol | 2017 Sep | 28774846 |
Nutrients Turned into Toxins: Microbiota Modulation of Nutrient Properties in Chronic Kidney Disease. | Nutrients | 2017 May 12 | 28498348 |
The influence of a chronic L-carnitine administration on the plasma metabolome ofmale Fischer 344 rats. | Mol Nutr Food Res | 2017 May | 27935219 |
Attenuation of Carbon Tetrachloride-Induced Hepatic Toxicity by a DietarySupplement. | J Diet Suppl | 2017 Mar 4 | 27471884 |
Effects of Combined Treatment with Branched-Chain Amino Acids, Citric Acid,L-Carnitine, Coenzyme Q10, Zinc, and Various Vitamins in Tumor-Bearing Mice. | Biol Pharm Bull | 2017 Mar 1 | 28123134 |
Urinary Excretion of Tetrodotoxin Modeled in a Porcine Renal Proximal Tubule Epithelial Cell Line, LLC-PK₁. | Mar Drugs | 2017 Jul 17 | 28714912 |
The effect of fermented buckwheat on producing l-carnitine- and γ-aminobutyricacid (GABA)-enriched designer eggs. | J Sci Food Agric | 2017 Jul | 27790703 |
Modulatory role of L-carnitine against microcystin-LR-induced immunotoxicity and oxidative stress in common carp. | Fish Physiol Biochem | 2017 Aug | 28315160 |
Fluorescent derivatization combined with aqueous solvent-based dispersive liquid-liquid microextraction for determination of butyrobetaine, l-carnitine and acetyl-l-carnitine in human plasma. | J Chromatogr A | 2016 Sep 16 | 27562416 |
l-carnitine protects rat hepatocytes from oxidative stress induced by T-2 toxin. | Drug Chem Toxicol | 2016 Oct | 26888052 |
Intense Exercise and Aerobic Conditioning Associated with Chromium or L-CarnitineSupplementation Modified the Fecal Microbiota of Fillies. | PLoS One | 2016 Dec 9 | 27935992 |
Comparison of the effect of a combination of eight micronutrients versus astandard mono preparation on sperm parameters. | Reprod Biol Endocrinol | 2016 Dec 9 | 27938385 |
Trimethylamine N-Oxide From Gut Microbiota in Chronic Kidney Disease Patients: Focus on Diet. | J Ren Nutr | 2015 Nov | 26235933 |
L-carnitine is a calcium chelator: a reason for its useful and toxic effects in biological systems. | J Basic Clin Physiol Pharmacol | 2015 Mar | 25046310 |
Alterations in Hepatic FGF21, Co-Regulated Genes, and Upstream Metabolic Genes in Response to Nutrition, Ketosis and Inflammation in Peripartal Holstein Cows. | PLoS One | 2015 | 26451842 |
Targets
- General Function:
- Xanthine oxidase 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 Da
References
- 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. [8255367 ]
- General Function:
- Receptor binding
- 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:
- 70857.055 Da
- Mechanism of Action:
- 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.
References
- 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. [12526798 ]
- General Function:
- Carnitine o-palmitoyltransferase 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:
- 88366.92 Da
- Mechanism of Action:
- 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.
References
- 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. [12705401 ]
- General Function:
- Carnitine o-palmitoyltransferase activity
- Gene Name:
- CPT2
- Uniprot ID:
- P23786
- Molecular Weight:
- 73776.335 Da
- Mechanism of Action:
- 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.
References
- 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. [12408750 ]
- General Function:
- Organic cation transmembrane 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:
- 64613.58 Da
References
- 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. [12089149 ]
- General Function:
- Symporter 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:
- 62154.48 Da
- Mechanism of Action:
- 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.
References
- 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. [12181285 ]
- Specific Function:
- Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.
- Gene Name:
- SLC25A20
- Uniprot ID:
- O43772
- Molecular Weight:
- 32943.46 Da
- Mechanism of Action:
- 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.
References
- Sekoguchi E, Sato N, Yasui A, Fukada S, Nimura Y, Aburatani H, Ikeda K, Matsuura A: A novel mitochondrial carnitine-acylcarnitine translocase induced by partial hepatectomy and fasting. J Biol Chem. 2003 Oct 3;278(40):38796-802. Epub 2003 Jul 25. [12882971 ]
- General Function:
- Triglyceride lipase activity
- 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 Da
References
- 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. [6509906 ]
- General Function:
- Acyl carnitine transmembrane transporter activity
- Specific Function:
- Transports arginine, lysine, homoarginine, methylarginine and, to a much lesser extent, ornithine and histidine. Does not transport carnitine nor acylcarnitines. Functions by both counter-exchange and uniport mechanisms.
- Gene Name:
- SLC25A29
- Uniprot ID:
- Q8N8R3
- Molecular Weight:
- 32061.87 Da
- Mechanism of Action:
- 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.
References
- Sekoguchi E, Sato N, Yasui A, Fukada S, Nimura Y, Aburatani H, Ikeda K, Matsuura A: A novel mitochondrial carnitine-acylcarnitine translocase induced by partial hepatectomy and fasting. J Biol Chem. 2003 Oct 3;278(40):38796-802. Epub 2003 Jul 25. [12882971 ]
- General Function:
- 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 Da
References
- 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. [16130180 ]
- General Function:
- Symporter 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:
- 62751.08 Da
- Mechanism of Action:
- 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.
References
- 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. [12175785 ]
- General Function:
- Receptor binding
- 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:
- 70177.935 Da
- Mechanism of Action:
- 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.
References
- 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. [15155769 ]