Human Metabolome Database Version 3.5

Showing metabocard for L-Carnitine (HMDB00062)

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
  • HMDB01467
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 Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (-)-(R)-3-Hydroxy-4-(trimethylammonio)butyrate
  2. (-)-carnitine
  3. (R)-(3-Carboxy-2-hydroxypropyl)trimethylammonium hydroxide
  4. (R)-carnitine
  5. (S)-carnitine
  6. 1-Carnitine
  7. 3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium
  8. 3-Hydroxy-4-trimethylammoniobutanoate
  9. 3-Hydroxy-4-trimethylammoniobutanoic acid
  10. Bicarnesine
  11. Carniking
  12. Carniking 50
  13. Carnilean
  14. Carnipass
  15. Carnipass 20
  16. Carnitene
  17. Carnitine
  18. Carnitor
  19. D-Carnitine
  20. delta-Carnitine
  21. DL-carnitine
  22. gamma-Trimethyl-ammonium-beta-hydroxybutirate
  23. gamma-Trimethyl-beta-hydroxybutyrobetaine
  24. gamma-Trimethyl-hydroxybutyrobetaine
  25. Karnitin
  26. L-(-)-Carnitine
  27. L-Carnitine
  28. L-gamma-Trimethyl-beta-hydroxybutyrobetaine
  29. Levocarnitina
  30. Levocarnitine
  31. Levocarnitinum
  32. R-(-)-3-Hydroxy-4-trimethylaminobutyrate
  33. 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)
Substituents
  • Beta Hydroxy Acid
  • Carboxylic Acid Salt
  • Choline
  • Secondary Alcohol
Direct Parent Carnitines
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Alanine and aspartate metabolism
  • Component of Fatty acid metabolism
  • Essential amino acid
Application Not Available
Cellular locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
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
Property Value Source
Water Solubility 5.33 g/L ALOGPS
LogP -2.90 ALOGPS
LogP -4.9 ChemAxon
LogS -1.60 ALOGPS
pKa (strongest acidic) 4.2 ChemAxon
pKa (strongest basic) -3.6 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 60.36 A2 ChemAxon
Rotatable Bond Count 4 ChemAxon
Refractivity 63.49 ChemAxon
Polarizability 16.9 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Muscle
  • Skeletal Muscle
  • Bladder
  • Fibroblasts
  • Intestine
  • Neuron
  • Testes
  • Erythrocyte
  • Kidney
  • Liver
  • Brain
  • Prostate
  • Adipose Tissue
  • Lung
  • Nerve Cells
  • Platelet
  • Myocardium
  • Sperm
Pathways
Name SMPDB Link KEGG Link
Beta Oxidation of Very Long Chain Fatty Acids SMP00052 map01040 Link_out
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
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
29.74 +/- 7.55 uM Adult (>18 years old) Not Specified Normal
Blood Detected and Quantified
33.6 +/- 6.2 uM Children (1-13 year old) Both Normal
  • Geigy Scient...
Blood Detected and Quantified
38.2 +/- 5.4 uM Adult (>18 years old) Female Normal
  • Geigy Scient...
Blood Detected and Quantified
43.0 (26.0-79.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
45.7 +/- 11.6 uM Adult (>18 years old) Not Specified Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
1.900 +/- 0.474 uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
4.0 (2.0 - 9.0) uM Adult (>18 years old) Both Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Normal
Urine Detected and Quantified
4.5 (0.62-15.2) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
5.0 (0.7-16.4) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
3.17 umol/mmol creatinine Adult (>18 years old) Male Normal
  • Shaykhutdino...
Urine Detected and Quantified
5.7 +/- 4.8 umol/mmol creatinine Adult (>18 years old) Female Normal
  • Geigy Scient...
Urine Detected and Quantified
23.5 +/- 4.0 umol/mmol creatinine Adult (>18 years old) Male Normal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
Urine Detected and Quantified
18.0 +/- 1.2 umol/mmol creatinine Adult (>18 years old) Female Normal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 41.7 +/- 23.9 uM Adult (>18 years old) Not Specified Heart Transplant
Urine Detected and Quantified 13.3 (11.6-15.1) umol/mmol creatinine Adult (>18 years old) Both Comment Diabetes
Urine Detected and Quantified 41.3 (38.3-44.2) umol/mmol creatinine Adult (>18 years old) Both Comment Diabetes
Urine Detected and Quantified 7.0 (0.0-30.0) umol/mmol creatinine Not Specified Both Lung cancer
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 Link_out
      Lung Cancer
      • 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. 2008 Oct 25. Pubmed: 18953024 Link_out
          Associated OMIM IDs
          DrugBank ID DB00583 Link_out
          DrugBank Metabolite ID Not Available
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB000572
          KNApSAcK ID Not Available
          Chemspider ID 2006614 Link_out
          KEGG Compound ID C15025 Link_out
          BioCyc ID CARNITINE Link_out
          BiGG ID 34600 Link_out
          Wikipedia Link L-Carnitine Link_out
          NuGOwiki Link HMDB00062 Link_out
          Metagene Link HMDB00062 Link_out
          METLIN ID 52 Link_out
          PubChem Compound 2724480 Link_out
          PDB ID 1NDF Link_out
          ChEBI ID 11060 Link_out
          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. 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 Link_out
          2. Evans AM, Fornasini G: Pharmacokinetics of L-carnitine. Clin Pharmacokinet. 2003;42(11):941-67. Pubmed: 12908852 Link_out
          3. 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 Link_out
          4. 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 Link_out
          5. 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 Link_out
          6. 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 Link_out
          7. 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 Link_out
          8. 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 Link_out
          9. 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 Link_out
          10. 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 Link_out
          11. 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 Link_out
          12. 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 Link_out
          13. 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 Link_out
          14. 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 Link_out
          15. 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 Link_out
          16. 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 Link_out
          17. Sinclair C, Gilchrist JM, Hennessey JV, Kandula M: Muscle carnitine in hypo- and hyperthyroidism. Muscle Nerve. 2005 Sep;32(3):357-9. Pubmed: 15803480 Link_out
          18. Ahmad S: L-carnitine in dialysis patients. Semin Dial. 2001 May-Jun;14(3):209-17. Pubmed: 11422928 Link_out
          19. 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 Link_out
          20. 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 Link_out
          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 Link_out
          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 Link_out

          Enzymes
          Name: Carnitine O-acetyltransferase
          Reactions:
          Acetyl-CoA + L-Carnitine unknown Coenzyme A + L-Acetylcarnitine details
          Gene Name: CRAT
          Uniprot ID: P43155 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Xanthine dehydrogenase/oxidase
          Reactions: Not Available
          Gene Name: XDH
          Uniprot ID: P47989 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Myeloperoxidase
          Reactions: Not Available
          Gene Name: MPO
          Uniprot ID: P05164 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Peroxisomal carnitine O-octanoyltransferase
          Reactions:
          Octanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Octanoylcarnitine details
          Gene Name: CROT
          Uniprot ID: Q9UKG9 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 1, muscle isoform
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT1B
          Uniprot ID: Q92523 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 1, liver isoform
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT1A
          Uniprot ID: P50416 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 2, mitochondrial
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT2
          Uniprot ID: P23786 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Carnitine O-palmitoyltransferase 1, brain isoform
          Reactions:
          hexadecanoyl-CoA + L-Carnitine unknown Coenzyme A + L-Palmitoylcarnitine details
          Gene Name: CPT1C
          Uniprot ID: Q8TCG5 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Gamma-butyrobetaine dioxygenase
          Reactions:
          4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen unknown L-Carnitine + Succinic acid + CO(2) details
          4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen unknown L-Carnitine + Succinic acid + Carbon dioxide details
          Gene Name: BBOX1
          Uniprot ID: O75936 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Liver carboxylesterase 1
          Reactions: Not Available
          Gene Name: CES1
          Uniprot ID: P23141 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Mitochondrial carnitine/acylcarnitine carrier protein
          Reactions: Not Available
          Gene Name: SLC25A20
          Uniprot ID: O43772 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Mitochondrial carnitine/acylcarnitine carrier protein CACL
          Reactions: Not Available
          Gene Name: SLC25A29
          Uniprot ID: Q8N8R3 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Transporters
          Name: Solute carrier organic anion transporter family member 1B1
          Reactions: Not Available
          Gene Name: SLCO1B1
          Uniprot ID: Q9Y6L6 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Solute carrier family 22 member 5
          Reactions: Not Available
          Gene Name: SLC22A5
          Uniprot ID: O76082 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Solute carrier family 22 member 4
          Reactions: Not Available
          Gene Name: SLC22A4
          Uniprot ID: Q9H015 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Solute carrier family 22 member 16
          Reactions: Not Available
          Gene Name: SLC22A16
          Uniprot ID: Q86VW1 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA