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Carnitine information


Carnitine
Clinical data
AHFS/Drugs.comMicromedex Detailed Consumer Information
Routes of
administration
Oral, intravenous
ATC code
  • A16AA01 (WHO) (l-form)
Legal status
Legal status
  • US: OTC
Pharmacokinetic data
Bioavailability<10%
Protein bindingNone
Metabolismslightly [clarification needed]
ExcretionUrine (>95%)
Identifiers
IUPAC name
  • 3-hydroxy-4-(trimethylazaniumyl)butanoate
CAS Number
  • 406-76-8 checkY
  • R-(-)-: 541-15-1 checkY
PubChem CID
  • 288
DrugBank
  • DB00583 ☒N
ChemSpider
  • 282 checkY
UNII
  • S7UI8SM58A
  • R-(-)-: 0G389FZZ9M checkY
KEGG
  • C00318 checkY
ChEBI
  • CHEBI:17126 checkY
ChEMBL
  • ChEMBL172513 checkY
CompTox Dashboard (EPA)
  • DTXSID3022744 Edit this at Wikidata
ECHA InfoCard100.006.343 Edit this at Wikidata
Chemical and physical data
FormulaC7H15NO3
Molar mass161.201 g·mol−1
3D model (JSmol)
  • Interactive image
SMILES
  • C[N+](C)(C)CC(CC(=O)[O-])O
InChI
  • InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3 checkY
  • Key:PHIQHXFUZVPYII-UHFFFAOYSA-N checkY
 ☒NcheckY (what is this?)  (verify)

Carnitine is a quaternary ammonium compound involved in metabolism in most mammals, plants, and some bacteria.[1][2][3][4] In support of energy metabolism, carnitine transports long-chain fatty acids from the cytosol into mitochondria to be oxidized for free energy production, and also participates in removing products of metabolism from cells.[3] Given its key metabolic roles, carnitine is concentrated in tissues like skeletal and cardiac muscle that metabolize fatty acids as an energy source.[3] Generally individuals, including strict vegetarians, synthesize enough L-carnitine in vivo.[1]

Carnitine exists as one of two stereoisomers: the two enantiomers d-carnitine (S-(+)-) and l-carnitine (R-(−)-).[5] Both are biologically active, but only l-carnitine naturally occurs in animals, and d-carnitine is toxic as it inhibits the activity of the l-form.[6] At room temperature, pure carnitine is a whiteish powder, and a water-soluble zwitterion with relatively low toxicity. Derived from amino acids,[7] carnitine was first extracted from meat extracts in 1905, leading to its name from Latin, "caro/carnis" or flesh.[2]

Some individuals with genetic or medical disorders (such as preterm infants) cannot make enough carnitine, requiring dietary supplementation.[1][3][4] Despite common carnitine supplement consumption among athletes for improved exercise performance or recovery, there is insufficient high-quality clinical evidence to indicate it provides any benefit.[3][4]

  1. ^ a b c "L-Carnitine". Micronutrient Information Center, Linus Pauling Institute, Oregon State University, Corvallis, OR. 2019-12-01. Retrieved 2020-04-29.
  2. ^ a b Bremer J (October 1983). "Carnitine--metabolism and functions". Physiological Reviews. 63 (4): 1420–80. doi:10.1152/physrev.1983.63.4.1420. PMID 6361812.
  3. ^ a b c d e "Carnitine". Office of Dietary Supplements, US National Institutes of Health. 2017-10-10. Retrieved 2020-04-29.
  4. ^ a b c "L-carnitine: Uses, benefits and dosage". Drugs.com. 2020-01-20. Retrieved 2020-04-29.
  5. ^ "Levocarnitine". PubChem, National Library of Medicine, US National Institutes of Health. 2020-04-25. Retrieved 2020-04-29.
  6. ^ Matsuoka M, Igisu H (July 1993). "Comparison of the effects of L-carnitine, D-carnitine and acetyl-L-carnitine on the neurotoxicity of ammonia". Biochemical Pharmacology. 46 (1): 159–64. doi:10.1016/0006-2952(93)90360-9. PMID 8347126.
  7. ^ Cox RA, Hoppel CL (December 1973). "Biosynthesis of carnitine and 4-N-trimethylaminobutyrate from 6-N-trimethyl-lysine". The Biochemical Journal. 136 (4): 1083–90. doi:10.1042/bj1361083. PMC 1166060. PMID 4786530.

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Carnitine

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Carnitine is a quaternary ammonium compound involved in metabolism in most mammals, plants, and some bacteria. In support of energy metabolism, carnitine...

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Acetylcarnitine

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Acetyl-L-carnitine, ALCAR or ALC, is an acetylated form of L-carnitine. It is naturally produced by the human body, and it is available as a dietary supplement...

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Carnitine palmitoyltransferase I

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Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase...

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Carnitine palmitoyltransferase II deficiency

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Carnitine palmitoyltransferase II deficiency, sometimes shortened to CPT-II or CPT2, is an autosomal recessively inherited genetic metabolic disorder...

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Carnitine palmitoyltransferase II

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Carnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene. Carnitine palmitoyltransferase II precursor...

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Systemic primary carnitine deficiency

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primary carnitine deficiency (SPCD) is an inborn error of fatty acid transport caused by a defect in the transporter responsible for moving carnitine across...

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Carnitine palmitoyltransferase I deficiency

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Carnitine palmitoyltransferase I deficiency is a rare metabolic disorder that prevents the body from converting certain fats called long-chain fatty acids(LCFA)...

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Carnitine biosynthesis

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Carnitine biosynthesis is a method for the endogenous production of L-carnitine, a molecule that is essential for energy metabolism. In humans and many...

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Beta oxidation

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membranes. Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine is converted back...

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Meldonium

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co-A is then exchanged with carnitine (via the enzyme carnitine palmitoyltransferase I) to produce a fatty acid-carnitine complex. This complex is then...

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Lysine

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polypeptides, uptake of essential mineral nutrients, and in the production of carnitine, which is key in fatty acid metabolism. Lysine is also often involved...

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B vitamins

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Vitamin B4: can refer to the distinct chemicals choline, adenine, or carnitine. Choline is synthesized by the human body, but not sufficiently to maintain...

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Inborn error of lipid metabolism

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carnitine deficiency - SLC22A5 (carnitine transporter) Carnitine-acylcarnitine translocase deficiency - Carnitine-acylcarnitine translocase Carnitine...

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Carnitine decarboxylase

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The enzyme carnitine decarboxylase (EC 4.1.1.42) catalyzes the chemical reaction carnitine ⇌ {\displaystyle \rightleftharpoons } 2-methylcholine + CO2...

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Pivmecillinam

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carnitine. This is not due to the drug itself, but to the pivalate anion, which is mostly removed from the body by forming a conjugate with carnitine...

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Glutaric aciduria type 1

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movement. GA1 causes secondary carnitine deficiency, as glutaric acid, like other organic acids, is detoxified by carnitine. Mental retardation may occur...

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Carnitine dehydratase

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In enzymology, a carnitine dehydratase (EC 4.2.1.89) is an enzyme that catalyzes the chemical reaction L-carnitine ⇌ {\displaystyle \rightleftharpoons...

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Acetyl group

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Ames, B. N. (2004). "Comparison of the effects of L-carnitine and acetyl-L-carnitine on carnitine levels, ambulatory activity, and oxidative stress biomarkers...

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Trimethylamine

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several routes in nature. Well studied are the degradation of choline and carnitine. Trimethylamine is used in the synthesis of choline, tetramethylammonium...

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