| Item |
Information |
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Drug Groups
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approved |
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Description
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Constituent of striated muscle and liver. It is used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias. [PubChem] |
| Indication |
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. |
| Pharmacology |
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. Lack of carnitine can lead to liver, heart, and muscle problems. Carnitine deficiency is defined biochemically as abnormally low plasma concentrations of free carnitine, less than 20 µmol/L at one week post term and may be associated with low tissue and/or urine concentrations. Further, this condition may be associated with a plasma concentration ratio of acylcarnitine/levocarnitine greater than 0.4 or abnormally elevated concentrations of acylcarnitine in the urine. Only the L isomer of carnitine (sometimes called vitamin BT) affects lipid metabolism. The "vitamin BT" form actually contains D,L-carnitine, which competitively inhibits levocarnitine and can cause deficiency. Levocarnitine can be used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias. |
| Toxicity |
LD50 > 8g/kg (mouse, oral). Adverse effects include hypertension, fever, tachycardia and seizures. |
| Affected Organisms |
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Humans and other mammals |
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| Biotransformation |
Major metabolites include trimethylamine N-oxide and [3H]-gamma-butyrobetaine. |
| Absorption |
Absolute bioavailability is 15% (tablets or solution). |
| Half Life |
17.4 hours (elimination) following a single intravenous dose. |
| Protein Binding |
None |
| References |
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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]
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| • |
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]
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| External Links |
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