Carnitine shuttle
 |
|
 |
|
| Clinical data | |
|---|---|
| AHFS/Drugs.com | Micromedex Detailed Consumer Information |
| Pregnancy category |
|
| Routes of administration |
Oral, intravenous |
| ATC code | |
| Legal status | |
| Legal status |
|
| Pharmacokinetic data | |
| Bioavailability | <10% |
| Protein binding | None |
| Metabolism | slightly |
| Excretion | Urine (>95%) |
| Identifiers | |
|
|
| CAS Number | |
| PubChem CID | |
| DrugBank | |
| ChemSpider | |
| UNII | |
| KEGG | |
| ChEBI | |
| ChEMBL | |
| Chemical and physical data | |
| Formula | C7H15NO3 |
| Molar mass | 161.199 g/mol |
| 3D model (JSmol) | |
|
|
|
|
  (what is this?)
|
|
Carnitine (β-hydroxy-γ-N-trimethylaminobutyric acid, 3-hydroxy-4-N,N,N- trimethylaminobutyrate) is a quaternary ammonium compound involved in metabolism in most mammals, plants and some bacteria. Carnitine may exist in two isomers, labeled D-carnitine and L-carnitine, as they are optically active. At room temperature, pure carnitine is a white powder, and a water-soluble zwitterion with low toxicity. Carnitine only exists in animals as the L-enantiomer, and D-carnitine is toxic because it inhibits the activity of L-carnitine. Carnitine was discovered in 1905 as a result of its high concentration in muscle tissue. It was originally labeled vitamin BT; however, because carnitine is synthesized in the human body, it is no longer considered a vitamin. Carnitine can be synthesized by most humans; about 1 in 350 males is unable to synthesize it due to genetic causes on the X chromosome. Carnitine is involved in the oxidation of fatty acids, and involved in systemic primary carnitine deficiency. It has been studied for preventing and treating other conditions, and is used as a purported performance enhancing drug.
Many eukaryotes have the ability to synthesize carnitine, including humans. Humans synthesize carnitine from the substrate TML (6-N-trimethyllysine), which is in turn derived from the methylation of the amino acid lysine. TML is then hydroxylated into hydroxytrimethyllysine (HTML) by trimethyllysine dioxygenase, requiring the presence of ascorbic acid. HTML is then cleaved by HTML aldose, yielding 4-trimethylaminobutyraldehyde (TMABA) and glycine. TMABA is then dehydrogenated into gamma-butyrobetaine, in an NAD+-dependent reaction, catalyzed by TMABA dehydrogenase. Gamma-butyrobetaine is then hydroxylated by gamma butyrobetaine hydroxylase into L-carnitine, requiring iron in the form of Fe2+.
...
Wikipedia