| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-04-14 16:29:59 -0600 |
| HMDB ID |
HMDB00062 |
| Secondary Accession Numbers |
|
| Metabolite Identification |
|---|
| Common Name |
L-Carnitine |
| 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." (http://www.dcnutrition.com). |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
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| Synonyms |
- (-)-(R)-3-Hydroxy-4-(trimethylammonio)butyrate
- (-)-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-Hydroxy-4-trimethylammoniobutanoate
- 3-Hydroxy-4-trimethylammoniobutanoic acid
- Bicarnesine
- 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-Carnitine
- L-gamma-Trimethyl-beta-hydroxybutyrobetaine
- Levocarnitina
- Levocarnitine
- Levocarnitinum
- R-(-)-3-Hydroxy-4-trimethylaminobutyrate
- Vitamin BT
|
| Chemical Formula |
C7H15NO3 |
| Average Molecular Weight |
161.1989 |
| Monoisotopic Molecular Weight |
161.105193351 |
| IUPAC Name |
(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate |
| Traditional IUPAC Name |
L-carnitine |
| CAS Registry Number |
541-15-1 |
| 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 Key |
PHIQHXFUZVPYII-ZCFIWIBFSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Aliphatic Acyclic Compounds |
| Class |
Alkylamines |
| Sub Class |
Quaternary Ammonium Salts |
| Other Descriptors |
- Aliphatic Acyclic Compounds
- a D,L-carnitine(Cyc)
- amino-acid betaine(ChEBI)
- gamma-amino acid(ChEBI)
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| Substituents |
- Beta Hydroxy Acid
- Carboxylic Acid Salt
- Choline
- Secondary Alcohol
|
| Direct Parent |
Carnitines |
| Ontology |
|---|
| Status |
Detected and Quantified |
| Origin |
|
| Biofunction |
- Component of Alanine and aspartate metabolism
- Component of Fatty acid metabolism
- Essential amino acid
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| Application |
Not Available |
| Cellular locations |
- Cytoplasm
- Extracellular
- Mitochondria
- Endoplasmic reticulum
- Peroxisome
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| Physical Properties |
|---|
| State |
Solid |
| Experimental Properties |
| Property |
Value |
Reference |
| Melting Point |
197 °C |
Not Available |
| Boiling Point |
Not Available |
Not Available |
| Water Solubility |
Not Available |
Not Available |
| LogP |
Not Available |
Not Available |
|
| Predicted Properties |
|
| Spectra |
|---|
|
Not Available
|
| Biological Properties |
|---|
| Cellular Locations |
- Cytoplasm
- Extracellular
- Mitochondria
- Endoplasmic reticulum
- Peroxisome
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| Biofluid Locations |
- Blood
- Cerebrospinal Fluid (CSF)
- Urine
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| Tissue Location |
- Muscle
- Skeletal Muscle
- Bladder
- Fibroblasts
- Intestine
- Neuron
- Testes
- Erythrocyte
- Kidney
- Liver
- Brain
- Prostate
- Adipose Tissue
- Lung
- Nerve Cells
- Platelet
- Myocardium
- Sperm
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| Pathways |
| Name |
SMPDB Link |
KEGG Link |
| Beta Oxidation of Very Long Chain Fatty Acids |
SMP00052
|
map01040 
|
| Oxidation of Branched Chain Fatty Acids |
SMP00030
|
Not Available
|
| Carnitine Synthesis |
SMP00465
|
Not Available
|
| Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids |
SMP00480
|
Not Available
|
| Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids |
SMP00482
|
Not Available
|
|
| Normal Concentrations |
|---|
|
| Blood |
Detected and Quantified |
|
29.74 +/- 7.55 uM |
Adult (>18 years old) |
Not Specified |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
33.6 +/- 6.2 uM |
Children (1-13 year old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
38.2 +/- 5.4 uM |
Adult (>18 years old) |
Female |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
43.0 (26.0-79.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
45.7 +/- 11.6 uM |
Adult (>18 years old) |
Not Specified |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
1.900 +/- 0.474 uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
4.0 (2.0 - 9.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and not Quantified |
|
Not Applicable |
Adult (>18 years old) |
Male |
Normal |
Not Available |
| Urine |
Detected and not Quantified |
|
Not Applicable |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
4.5 (0.62-15.2) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
by DFI-MS/MS (Biocrates kit)
|
| Urine |
Detected and Quantified |
|
5.0 (0.7-16.4) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
urine by NMR
|
| Urine |
Detected and Quantified |
|
3.17 umol/mmol creatinine |
Adult (>18 years old) |
Male |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
23.5 +/- 4.0 umol/mmol creatinine |
Adult (>18 years old) |
Male |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
18.0 +/- 1.2 umol/mmol creatinine |
Adult (>18 years old) |
Female |
Normal |
Not Available |
|
| Abnormal Concentrations |
|---|
|
| Blood |
Detected and Quantified |
|
41.7 +/- 23.9 uM |
Adult (>18 years old) |
Not Specified |
Heart Transplant |
Not Available |
| Urine |
Detected and Quantified |
|
13.3 (11.6-15.1) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Diabetes |
With ketoacidosis,
|
| Urine |
Detected and Quantified |
|
41.3 (38.3-44.2) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Diabetes |
With ketoacidosis
|
| Urine |
Detected and Quantified |
|
7.0 (0.0-30.0) umol/mmol creatinine |
Not Specified |
Both |
Lung cancer |
Not Available |
|
| Associated Disorders and Diseases |
|---|
| Disease References |
| Diabetes mellitus type 2 |
|---|
- 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
|
| Lung Cancer |
|---|
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| Associated OMIM IDs |
|
| External Links |
|---|
| DrugBank ID |
DB00583 |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB000572 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
2006614 |
| KEGG Compound ID |
C15025 |
| BioCyc ID |
CARNITINE |
| BiGG ID |
34600 |
| Wikipedia Link |
L-Carnitine |
| NuGOwiki Link |
HMDB00062 |
| Metagene Link |
HMDB00062 |
| METLIN ID |
52 |
| PubChem Compound |
2724480 |
| PDB ID |
1NDF |
| ChEBI ID |
11060 |
| 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 |
- 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
- Evans AM, Fornasini G: Pharmacokinetics of L-carnitine. Clin Pharmacokinet. 2003;42(11):941-67.
Pubmed: 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.
Pubmed: 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.
Pubmed: 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.
Pubmed: 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.
Pubmed: 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.
Pubmed: 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.
Pubmed: 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.
Pubmed: 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.
Pubmed: 16227137
- Lerch R: [The effect of L-carnitine on ischemic heart disease: experimental results] Schweiz Rundsch Med Prax. 1998 Jan 21;87(4):97-100.
Pubmed: 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.
Pubmed: 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.
Pubmed: 10094444
- 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
- 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
- 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
- Sinclair C, Gilchrist JM, Hennessey JV, Kandula M: Muscle carnitine in hypo- and hyperthyroidism. Muscle Nerve. 2005 Sep;32(3):357-9.
Pubmed: 15803480
- Ahmad S: L-carnitine in dialysis patients. Semin Dial. 2001 May-Jun;14(3):209-17.
Pubmed: 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.
Pubmed: 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.
Pubmed: 19212411
|
