Identification

Name
L-Carnitine
Accession Number
DB00583  (APRD01070)
Type
Small Molecule
Groups
Approved
Description

Constituent of striated muscle and liver. It is used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias.

Structure
Thumb
Synonyms
  • (-)-Carnitine
  • (-)-L-Carnitine
  • (R)-Carnitine
  • 3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium hydroxide, inner salt
  • Carnitine
  • Levocarnitin
  • Levocarnitina
  • Lévocarnitine
  • Levocarnitine
  • Levocarnitinum
  • Vitamin BT
External IDs
ST 261
Product Ingredients
IngredientUNIICASInChI Key
L-Carnitin HydrochlorideNot AvailableNot AvailableNot applicable
Product Images
Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
CarnitorTablet330 mg/1OralSigma Tau Pharmaceuticals, Inc.1985-12-27Not applicableUs50383 0172 90 nlmimage10 363f9b2c
CarnitorSolution1 g/10mLOralSigma Tau Pharmaceuticals, Inc.1986-04-10Not applicableUs
CarnitorInjection, solution1 g/5mLIntravenousSigma Tau Pharmaceuticals, Inc.1992-12-16Not applicableUs
Carnitor - Liq IV 200mg/mlSolution200 mgIntravenousSigma Tau Pharmaceuticals, Inc.1995-12-31Not applicableCanada
Carnitor - Liq Orl 100mg/mlLiquid100 mgOralSigma Tau Pharmaceuticals, Inc.1995-12-31Not applicableCanada
Carnitor -tab 330mgTablet330 mgOralSigma Tau Pharmaceuticals, Inc.1995-12-31Not applicableCanada
Carnitor SFSolution1 g/10mLOralSigma Tau Pharmaceuticals, Inc.1986-04-10Not applicableUs
LevocarnitineTablet330 mg/1OralHi Tech Pharmacal Co., Inc.2005-11-01Not applicableUs
LevocarnitineSolution1 g/10mLOralHi Tech Pharmacal Co., Inc.1986-04-10Not applicableUs
LevocarnitineTablet330 mg/1OralAvera Mc Kennan Hospital2016-07-12Not applicableUs
Generic Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
LevocarnitineInjection1 g/5mLIntravenousTeva Parenteral Medicines, Inc.2001-06-012017-03-01Us
LevocarnitineSolution1 g/10mLOralRising Pharmaceuticals2004-09-20Not applicableUs
LevocarnitineInjection2.5 g/12.5mLIntravenousTeva Parenteral Medicines, Inc.2001-06-012017-03-01Us
LevocarnitineInjection, solution200 mg/mLIntravenousAmerican Regent2001-10-08Not applicableUs
LevocarnitineSolution1 g/10mLOralRemedy Repack2013-10-082016-12-29Us
LevocarnitineTablet330 mg/1OralRising Pharmaceuticals2004-09-202017-12-31Us
International/Other Brands
Albicar (Casasco) / Aveptol (Leovan Pharmaceuticals) / Biocarn (Medice) / Bitobionil (S.J.A.) / Cardimax (Integrated) / Cardispan (Grossman) / Carniben (Bennett Pharmaceuticals) / Carnicor (Sigma Tau) / Carnitene / Carnitor / Lecarna (Bennett Pharmaceuticals)
Categories
UNII
0G389FZZ9M
CAS number
541-15-1
Weight
Average: 161.1989
Monoisotopic: 161.105193351
Chemical Formula
C7H15NO3
InChI Key
PHIQHXFUZVPYII-ZCFIWIBFSA-N
InChI
InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/t6-/m1/s1
IUPAC Name
(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate
SMILES
C[N+](C)(C)C[[email protected]](O)CC([O-])=O

Pharmacology

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.

Structured Indications
Pharmacodynamics

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.

Mechanism of action

Levocarnitine can be synthesised within the body from the amino acids lysine or methionine. Vitamin C (ascorbic acid) is essential to the synthesis of carnitine. 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. Only the L isomer of carnitine (sometimes called vitamin BT) affects lipid metabolism. Levocarnitine is handled by several proteins in different pathways including carnitine transporters, carnitine translocases, carnitine acetyltransferases and carnitine palmitoyltransferases.

TargetActionsOrganism
UCarnitine O-palmitoyltransferase 1, liver isoform
activator
Human
USolute carrier family 22 member 4Not AvailableHuman
USolute carrier family 22 member 5Not AvailableHuman
UCarnitine O-acetyltransferaseNot AvailableHuman
UMitochondrial carnitine/acylcarnitine carrier protein CACLNot AvailableHuman
UMitochondrial carnitine/acylcarnitine carrier proteinNot AvailableHuman
UPeroxisomal carnitine O-octanoyltransferaseNot AvailableHuman
UCarnitine O-palmitoyltransferase 2, mitochondrialNot AvailableHuman
UXanthine dehydrogenase/oxidaseNot AvailableHuman
ULiver carboxylesterase 1Not AvailableHuman
UMyeloperoxidaseNot AvailableHuman
Absorption

Absolute bioavailability is 15% (tablets or solution). Time to maximum plasma concentration was found to be 3.3 hours.

Volume of distribution

The steady state volume of distribution (Vss) of an intravenously administered dose, above endogenous baseline levels, was calculated to be 29.0 +/- 7.1L. However this value is predicted to be an underestimate of the true Vss.

Protein binding

None

Metabolism

After oral administration L-carnitine which is unabsorbed is metabolized in the gastrointestinal tract by bacterial microflora. Major metabolites include trimethylamine N-oxide and [3H]-gamma-butyrobetaine.

Route of elimination

Following a single intravenous dose, 73.1 +/- 16% of the dose was excreted in the urine during the 0-24 hour interval. Post administration of oral carnitine supplements, in addition to a high carnitine diet, 58-65% of the administered radioactive dose was recovered from urine and feces in 5-11 days.

Half life

17.4 hours (elimination) following a single intravenous dose.

Clearance

Total body clearance was found to be a mean of 4L/h.

Toxicity

LD50 > 8g/kg (mouse, oral). Adverse effects include hypertension, fever, tachycardia and seizures.

Affected organisms
  • Humans and other mammals
Pathways
PathwayCategory
Trifunctional protein deficiencyDisease
Glutaric Aciduria Type IDisease
Very-long-chain acyl coa dehydrogenase deficiency (VLCAD)Disease
Carnitine SynthesisMetabolic
Carnitine-acylcarnitine translocase deficiencyDisease
Long chain acyl-CoA dehydrogenase deficiency (LCAD)Disease
Oxidation of Branched Chain Fatty AcidsMetabolic
Fatty acid MetabolismMetabolic
Beta Oxidation of Very Long Chain Fatty AcidsMetabolic
Ethylmalonic EncephalopathyDisease
Carnitine palmitoyl transferase deficiency (II)Disease
Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency)Disease
Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty AcidsMetabolic
Adrenoleukodystrophy, X-linkedDisease
Carnitine palmitoyl transferase deficiency (I)Disease
Medium chain acyl-coa dehydrogenase deficiency (MCAD)Disease
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
DrugInteractionDrug group
AcenocoumarolL-Carnitine may increase the anticoagulant activities of Acenocoumarol.Approved
ClorindioneL-Carnitine may increase the anticoagulant activities of Clorindione.Experimental
DicoumarolL-Carnitine may increase the anticoagulant activities of Dicoumarol.Approved
DiphenadioneL-Carnitine may increase the anticoagulant activities of Diphenadione.Experimental
Ethyl biscoumacetateL-Carnitine may increase the anticoagulant activities of Ethyl biscoumacetate.Withdrawn
FluindioneL-Carnitine may increase the anticoagulant activities of Fluindione.Investigational
PhenindioneL-Carnitine may increase the anticoagulant activities of Phenindione.Approved
PhenprocoumonL-Carnitine may increase the anticoagulant activities of Phenprocoumon.Approved
TioclomarolL-Carnitine may increase the anticoagulant activities of Tioclomarol.Experimental
WarfarinL-Carnitine may increase the anticoagulant activities of Warfarin.Approved
Food Interactions
Not Available

References

Synthesis Reference

Noguchi, J. and Sakota, N.; US. Patent 3,135,788; June 2,1964; assigned to Nihon Zoki Seiyaku Kabushikikaisha (Japan).

US4413142
General References
  1. 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]
  2. 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]
External Links
Human Metabolome Database
HMDB00062
KEGG Drug
D02176
KEGG Compound
C00318
PubChem Compound
10917
PubChem Substance
46505864
ChemSpider
10455
BindingDB
50037268
ChEBI
16347
ChEMBL
CHEMBL1149
Therapeutic Targets Database
DAP000958
PharmGKB
PA450154
RxList
RxList Drug Page
Wikipedia
L-Carnitine
ATC Codes
A16AA01 — Levocarnitine
AHFS Codes
  • 40:20.00 — Caloric Agents
MSDS
Download (72.7 KB)

Clinical Trials

Clinical Trials
PhaseStatusPurposeConditionsCount
0Enrolling by InvitationBasic ScienceDietary Modifications / Nutrition1
1RecruitingTreatmentAutism Spectrum Conditions/Disorders / Mitochondrial Diseases1
1TerminatedTreatmentShock, Septic1
1Unknown StatusTreatmentAtrioventricular Septal Defects / Heart Septal Defects, Ventricular1
1, 2Active Not RecruitingTreatmentDNA Damage / Endocrine Diseases / Fertility Decreased Male1
1, 2CompletedTreatmentSpinal Muscular Atrophy Type I1
2CompletedTreatmentAcquired Immune Deficiency Syndrome (AIDS) / Human Immunodeficiency Virus (HIV) Infections1
2CompletedTreatmentComplication of Hemodialysis1
2CompletedTreatmentEnd-Stage Renal Disease (ESRD)2
2CompletedTreatmentHuman Immunodeficiency Virus (HIV) Infections5
2CompletedTreatmentNeoplasms1
2CompletedTreatmentSpinal Cord Injuries (SCI)1
2CompletedTreatmentSpinal Muscular Atrophy (SMA)2
2CompletedTreatmentΒ-Thalassemia Intermedia1
2RecruitingTreatmentNon-Alcoholic Steatohepatitis1
2RecruitingTreatmentPolycystic Ovarian Syndrome2
2Unknown StatusTreatmentAttention Deficit Hyperactivity Disorder (ADHD)1
2Unknown StatusTreatmentHuman Immunodeficiency Virus (HIV) Infections1
2, 3CompletedPreventionHeart Failure, Unspecified1
2, 3CompletedTreatmentHeart Failure, Unspecified1
2, 3RecruitingPreventionCarnitine Deficiency / Neurodevelopmental Disorders / Premature Babies1
2, 3RecruitingTreatmentAmmonia Metabolism1
3CompletedPreventionNonvalvular Atrial Fibrillation1
3CompletedTreatmentComplication of Hemodialysis / Hyperthyroidism Treated or Under Control1
3CompletedTreatmentHuman Immunodeficiency Virus (HIV) Infections1
3CompletedTreatmentHuman Immunodeficiency Virus (HIV) Infections / Infections, Cytomegalovirus1
3RecruitingTreatmentSpinal Muscular Atrophy (SMA)1
3TerminatedPreventionAcute Kidney Injury (AKI) / Impaired Renal Function1
3Unknown StatusPreventionAcute Pyelonephritis(APN)1
3Unknown StatusTreatmentDisseminated Sclerosis1
3WithdrawnTreatmentPostpoliomyelitis Syndrome1
4CompletedHealth Services ResearchAcidosis, Respiratory / Acquired Immune Deficiency Syndrome (AIDS) / Human Immunodeficiency Virus (HIV) Infections / Lipodystrophies1
4CompletedTreatmentHypothyroidism1
4Unknown StatusNot AvailableCarnitine Deficiency1
4Unknown StatusTreatmentAnemias / Arterial Hypotension / Cardiac Complications / Muscle Weakness / Signs and Symptoms1
4Unknown StatusTreatmentTreatment Resistant Disorders1
Not AvailableCompletedNot AvailableDiabetes, Diabetes Mellitus Type 1 / Hypoglycemia1
Not AvailableCompletedPreventionComplications of Prematurity1
Not AvailableCompletedPreventionGlucose tolerance impaired1
Not AvailableCompletedPreventionLiver Cirrhosis1
Not AvailableCompletedTreatmentHuman Immunodeficiency Virus (HIV) Infections2
Not AvailableNot Yet RecruitingPreventionGlucose tolerance impaired1
Not AvailableWithdrawnTreatmentCrohn's Disease (CD) / Tiredness1

Pharmacoeconomics

Manufacturers
  • Sigma tau pharmaceuticals inc
  • Bedford laboratories div ben venue laboratories inc
  • Luitpold pharmaceuticals inc
  • Teva parenteral medicines inc
  • Hi tech pharmacal co inc
  • Lyne laboratories inc
  • Corepharma llc
Packagers
Dosage forms
FormRouteStrength
Injection, solutionIntravenous1 g/5mL
SolutionIntravenous200 mg
LiquidOral100 mg
TabletOral330 mg
InjectionIntravenous1 g/5mL
InjectionIntravenous2.5 g/12.5mL
Injection, solutionIntravenous200 mg/mL
SolutionOral1 g/10mL
TabletOral330 mg/1
Prices
Unit descriptionCostUnit
Carnitor 90 330 mg tablet Bottle90.44USD bottle
Carnitor 1 gm/10ml Solution 118ml Bottle39.99USD bottle
Carnitor 1 gm/5 ml vial7.68USD ml
L-carnitine powder4.28USD g
Carnitor 330 mg tablet0.97USD tablet
L-carnitine 500 mg caplet0.31USD caplet
G-levocarnitine 100 mg/ml soln0.15USD ml
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
Patents
Patent NumberPediatric ExtensionApprovedExpires (estimated)
US6335369No2001-01-182021-01-18Us
US6429230No2001-01-182021-01-18Us
US6696493No2001-01-182021-01-18Us

Properties

State
Solid
Experimental Properties
PropertyValueSource
melting point (°C)195-198Noguchi, J. and Sakota, N.; US. Patent 3,135,788; June 2,1964; assigned to Nihon Zoki Seiyaku Kabushikikaisha (Japan).
water solubility2500 mg/mLNot Available
pKa3.8Not Available
Predicted Properties
PropertyValueSource
Water Solubility5.33 mg/mLALOGPS
logP-2.9ALOGPS
logP-4.9ChemAxon
logS-1.6ALOGPS
pKa (Strongest Acidic)4.2ChemAxon
pKa (Strongest Basic)-3.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area60.36 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity63.49 m3·mol-1ChemAxon
Polarizability16.93 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted ADMET features
PropertyValueProbability
Human Intestinal Absorption-0.9409
Blood Brain Barrier+0.8568
Caco-2 permeable+0.5952
P-glycoprotein substrateNon-substrate0.6583
P-glycoprotein inhibitor INon-inhibitor0.9784
P-glycoprotein inhibitor IINon-inhibitor0.8966
Renal organic cation transporterNon-inhibitor0.9161
CYP450 2C9 substrateNon-substrate0.8372
CYP450 2D6 substrateNon-substrate0.845
CYP450 3A4 substrateNon-substrate0.5297
CYP450 1A2 substrateNon-inhibitor0.9361
CYP450 2C9 inhibitorNon-inhibitor0.951
CYP450 2D6 inhibitorNon-inhibitor0.9382
CYP450 2C19 inhibitorNon-inhibitor0.9348
CYP450 3A4 inhibitorNon-inhibitor0.9627
CYP450 inhibitory promiscuityLow CYP Inhibitory Promiscuity0.9874
Ames testNon AMES toxic0.8832
CarcinogenicityCarcinogens 0.6127
BiodegradationReady biodegradable0.9234
Rat acute toxicity2.1624 LD50, mol/kg Not applicable
hERG inhibition (predictor I)Weak inhibitor0.9731
hERG inhibition (predictor II)Non-inhibitor0.914
ADMET data is predicted using admetSAR, a free tool for evaluating chemical ADMET properties. (23092397)

Spectra

Mass Spec (NIST)
Not Available
Spectra
SpectrumSpectrum TypeSplash Key
Predicted GC-MS Spectrum - GC-MSPredicted GC-MSNot Available
Predicted MS/MS Spectrum - 10V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 20V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 40V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 10V, Negative (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 20V, Negative (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 40V, Negative (Annotated)Predicted LC-MS/MSNot Available
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-03di-0900000000-9b579c570aab7c7d3a21
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0ik9-0900000000-e37d3def2de1af5bd2f5
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0ue9-8900000000-cf37f0a721cd52d86e8e
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0r0c-9200000000-a11a4872036f3c33d25a
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0a4l-9000000000-2402e260a330ff1772a1
LC-MS/MS Spectrum - LC-ESI-IT , positiveLC-MS/MSsplash10-0w29-6900000000-d0136248acdd706e650b
LC-MS/MS Spectrum - LC-ESI-QTOF , positiveLC-MS/MSsplash10-0ik9-2900000000-61732bb10307f2b183ca
LC-MS/MS Spectrum - LC-ESI-QTOF , positiveLC-MS/MSsplash10-0zg0-9400000000-15c3db3324f1d46d3c7c
LC-MS/MS Spectrum - LC-ESI-QTOF , positiveLC-MS/MSsplash10-0ik9-2900000000-ee79f10dce9fc09246ec
1H NMR Spectrum1D NMRNot Applicable

Taxonomy

Description
This compound belongs to the class of organic compounds known as carnitines. These are organic compounds containing the quaternary ammonium compound carnitine.
Kingdom
Organic compounds
Super Class
Organic nitrogen compounds
Class
Organonitrogen compounds
Sub Class
Quaternary ammonium salts
Direct Parent
Carnitines
Alternative Parents
Short-chain hydroxy acids and derivatives / Beta hydroxy acids and derivatives / Fatty acids and conjugates / Tetraalkylammonium salts / Secondary alcohols / Carboxylic acid salts / 1,2-aminoalcohols / Monocarboxylic acids and derivatives / Carboxylic acids / Organopnictogen compounds
show 5 more
Substituents
Carnitine / Beta-hydroxy acid / Short-chain hydroxy acid / Fatty acid / Hydroxy acid / Tetraalkylammonium salt / 1,2-aminoalcohol / Carboxylic acid salt / Secondary alcohol / Carboxylic acid derivative
show 13 more
Molecular Framework
Aliphatic acyclic compounds
External Descriptors
carnitine (CHEBI:16347)

Targets

Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Activator
General Function
Carnitine o-palmitoyltransferase activity
Specific Function
Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-o...
Gene Name
CPT1A
Uniprot ID
P50416
Uniprot Name
Carnitine O-palmitoyltransferase 1, liver isoform
Molecular Weight
88366.92 Da
References
  1. Xu ZR, Wang MQ, Mao HX, Zhan XA, Hu CH: Effects of L-carnitine on growth performance, carcass composition, and metabolism of lipids in male broilers. Poult Sci. 2003 Mar;82(3):408-13. [PubMed:12705401]
  2. Morillas M, Lopez-Vinas E, Valencia A, Serra D, Gomez-Puertas P, Hegardt FG, Asins G: Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal. Biochem J. 2004 May 1;379(Pt 3):777-84. [PubMed:14711372]
  3. Tripodi G, Modica R, Stella A, Bigatti G, Bianchi G, Stella P: Haplotype analysis of carnitine transporters and left ventricular mass in human essential hypertension. J Ren Nutr. 2005 Jan;15(1):2-7. [PubMed:15647998]
  4. 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]
  5. Shin ES, Cho SY, Lee EH, Lee SJ, Chang IS, Lee TR: Positive regulation of hepatic carnitine palmitoyl transferase 1A (CPT1A) activities by soy isoflavones and L-carnitine. Eur J Nutr. 2006 Mar;45(3):159-64. Epub 2005 Dec 20. [PubMed:16362726]
  6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Symporter activity
Specific Function
Sodium-ion dependent, low affinity carnitine transporter. Probably transports one sodium ion with one molecule of carnitine. Also transports organic cations such as tetraethylammonium (TEA) without...
Gene Name
SLC22A4
Uniprot ID
Q9H015
Uniprot Name
Solute carrier family 22 member 4
Molecular Weight
62154.48 Da
References
  1. Kristufek D, Rudorfer W, Pifl C, Huck S: Organic cation transporter mRNA and function in the rat superior cervical ganglion. J Physiol. 2002 Aug 15;543(Pt 1):117-34. [PubMed:12181285]
  2. Amat di San Filippo C, Wang Y, Longo N: Functional domains in the carnitine transporter OCTN2, defective in primary carnitine deficiency. J Biol Chem. 2003 Nov 28;278(48):47776-84. Epub 2003 Sep 23. [PubMed:14506273]
  3. Lamhonwah AM, Ackerley C, Onizuka R, Tilups A, Lamhonwah D, Chung C, Tao KS, Tellier R, Tein I: Epitope shared by functional variant of organic cation/carnitine transporter, OCTN1, Campylobacter jejuni and Mycobacterium paratuberculosis may underlie susceptibility to Crohn's disease at 5q31. Biochem Biophys Res Commun. 2005 Dec 2;337(4):1165-75. Epub 2005 Oct 6. [PubMed:16246312]
  4. Lash LH, Putt DA, Cai H: Membrane transport function in primary cultures of human proximal tubular cells. Toxicology. 2006 Dec 7;228(2-3):200-18. Epub 2006 Sep 1. [PubMed:16997449]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Symporter activity
Specific Function
Sodium-ion dependent, high affinity carnitine transporter. Involved in the active cellular uptake of carnitine. Transports one sodium ion with one molecule of carnitine. Also transports organic cat...
Gene Name
SLC22A5
Uniprot ID
O76082
Uniprot Name
Solute carrier family 22 member 5
Molecular Weight
62751.08 Da
References
  1. Lahjouji K, Elimrani I, Wu J, Mitchell GA, Qureshi IA: A heterozygote phenotype is present in the jvs +/- mutant mouse livers. Mol Genet Metab. 2002 May;76(1):76-80. [PubMed:12175785]
  2. Kristufek D, Rudorfer W, Pifl C, Huck S: Organic cation transporter mRNA and function in the rat superior cervical ganglion. J Physiol. 2002 Aug 15;543(Pt 1):117-34. [PubMed:12181285]
  3. Ohashi R, Tamai I, Inano A, Katsura M, Sai Y, Nezu J, Tsuji A: Studies on functional sites of organic cation/carnitine transporter OCTN2 (SLC22A5) using a Ser467Cys mutant protein. J Pharmacol Exp Ther. 2002 Sep;302(3):1286-94. [PubMed:12183691]
  4. Hou JW: Primary systemic carnitine deficiency presenting as recurrent Reye-like syndrome and dilated cardiomyopathy. Chang Gung Med J. 2002 Dec;25(12):832-7. [PubMed:12635840]
  5. Friedrich A, Prasad PD, Freyer D, Ganapathy V, Brust P: Molecular cloning and functional characterization of the OCTN2 transporter at the RBE4 cells, an in vitro model of the blood-brain barrier. Brain Res. 2003 Apr 4;968(1):69-79. [PubMed:12644265]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Receptor binding
Specific Function
Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of ...
Gene Name
CRAT
Uniprot ID
P43155
Uniprot Name
Carnitine O-acetyltransferase
Molecular Weight
70857.055 Da
References
  1. Jogl G, Tong L: Crystal structure of carnitine acetyltransferase and implications for the catalytic mechanism and fatty acid transport. Cell. 2003 Jan 10;112(1):113-22. [PubMed:12526798]
  2. Wu D, Govindasamy L, Lian W, Gu Y, Kukar T, Agbandje-McKenna M, McKenna R: Structure of human carnitine acetyltransferase. Molecular basis for fatty acyl transfer. J Biol Chem. 2003 Apr 11;278(15):13159-65. Epub 2003 Jan 31. [PubMed:12562770]
  3. Vikramadithyan RK, Hiriyan J, Suresh J, Gershome C, Babu RK, Misra P, Rajagopalan R, Chakrabarti R: DRF 2655: a unique molecule that reduces body weight and ameliorates metabolic abnormalities. Obes Res. 2003 Feb;11(2):292-303. [PubMed:12582227]
  4. Govindasamy L, Kukar T, Lian W, Pedersen B, Gu Y, Agbandje-McKenna M, Jin S, McKenna R, Wu D: Structural and mutational characterization of L-carnitine binding to human carnitine acetyltransferase. J Struct Biol. 2004 Jun;146(3):416-24. [PubMed:15099582]
  5. Cordente AG, Lopez-Vinas E, Vazquez MI, Swiegers JH, Pretorius IS, Gomez-Puertas P, Hegardt FG, Asins G, Serra D: Redesign of carnitine acetyltransferase specificity by protein engineering. J Biol Chem. 2004 Aug 6;279(32):33899-908. Epub 2004 May 21. [PubMed:15155769]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Acyl carnitine transmembrane transporter activity
Specific Function
Transports arginine, lysine, homoarginine, methylarginine and, to a much lesser extent, ornithine and histidine. Does not transport carnitine nor acylcarnitines. Functions by both counter-exchange ...
Gene Name
SLC25A29
Uniprot ID
Q8N8R3
Uniprot Name
Mitochondrial basic amino acids transporter
Molecular Weight
32061.87 Da
References
  1. Sekoguchi E, Sato N, Yasui A, Fukada S, Nimura Y, Aburatani H, Ikeda K, Matsuura A: A novel mitochondrial carnitine-acylcarnitine translocase induced by partial hepatectomy and fasting. J Biol Chem. 2003 Oct 3;278(40):38796-802. Epub 2003 Jul 25. [PubMed:12882971]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Not Available
Specific Function
Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid...
Gene Name
SLC25A20
Uniprot ID
O43772
Uniprot Name
Mitochondrial carnitine/acylcarnitine carrier protein
Molecular Weight
32943.46 Da
References
  1. Sekoguchi E, Sato N, Yasui A, Fukada S, Nimura Y, Aburatani H, Ikeda K, Matsuura A: A novel mitochondrial carnitine-acylcarnitine translocase induced by partial hepatectomy and fasting. J Biol Chem. 2003 Oct 3;278(40):38796-802. Epub 2003 Jul 25. [PubMed:12882971]
  2. Peluso G, Petillo O, Margarucci S, Grippo P, Melone MA, Tuccillo F, Calvani M: Differential carnitine/acylcarnitine translocase expression defines distinct metabolic signatures in skeletal muscle cells. J Cell Physiol. 2005 May;203(2):439-46. [PubMed:15515015]
  3. Tonazzi A, Giangregorio N, Indiveri C, Palmieri F: Identification by site-directed mutagenesis and chemical modification of three vicinal cysteine residues in rat mitochondrial carnitine/acylcarnitine transporter. J Biol Chem. 2005 May 20;280(20):19607-12. Epub 2005 Mar 9. [PubMed:15757911]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Receptor binding
Specific Function
Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate. Converts the end product of pristanic acid beta oxidation, 4,8-dimethylnonanoyl-CoA, to its corres...
Gene Name
CROT
Uniprot ID
Q9UKG9
Uniprot Name
Peroxisomal carnitine O-octanoyltransferase
Molecular Weight
70177.935 Da
References
  1. Cordente AG, Lopez-Vinas E, Vazquez MI, Swiegers JH, Pretorius IS, Gomez-Puertas P, Hegardt FG, Asins G, Serra D: Redesign of carnitine acetyltransferase specificity by protein engineering. J Biol Chem. 2004 Aug 6;279(32):33899-908. Epub 2004 May 21. [PubMed:15155769]
  2. Cordente AG, Lopez-Vinas E, Vazquez MI, Gomez-Puertas P, Asins G, Serra D, Hegardt FG: Mutagenesis of specific amino acids converts carnitine acetyltransferase into carnitine palmitoyltransferase. Biochemistry. 2006 May 16;45(19):6133-41. [PubMed:16681386]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Carnitine o-palmitoyltransferase activity
Specific Function
Not Available
Gene Name
CPT2
Uniprot ID
P23786
Uniprot Name
Carnitine O-palmitoyltransferase 2, mitochondrial
Molecular Weight
73776.335 Da
References
  1. Barrero MJ, Camarero N, Marrero PF, Haro D: Control of human carnitine palmitoyltransferase II gene transcription by peroxisome proliferator-activated receptor through a partially conserved peroxisome proliferator-responsive element. Biochem J. 2003 Feb 1;369(Pt 3):721-9. [PubMed:12408750]
  2. Kong JY, Rabkin SW: Lovastatin does not accentuate but is rather additive to palmitate-induced apoptosis in cardiomyocytes. Prostaglandins Leukot Essent Fatty Acids. 2002 Nov;67(5):293-302. [PubMed:12445488]
  3. Rasmussen BB, Holmback UC, Volpi E, Morio-Liondore B, Paddon-Jones D, Wolfe RR: Malonyl coenzyme A and the regulation of functional carnitine palmitoyltransferase-1 activity and fat oxidation in human skeletal muscle. J Clin Invest. 2002 Dec;110(11):1687-93. [PubMed:12464674]
  4. Price NT, Jackson VN, van der Leij FR, Cameron JM, Travers MT, Bartelds B, Huijkman NC, Zammit VA: Cloning and expression of the liver and muscle isoforms of ovine carnitine palmitoyltransferase 1: residues within the N-terminus of the muscle isoform influence the kinetic properties of the enzyme. Biochem J. 2003 Jun 15;372(Pt 3):871-9. [PubMed:12662154]
  5. Lehtihet M, Welsh N, Berggren PO, Cook GA, Sjoholm A: Glibenclamide inhibits islet carnitine palmitoyltransferase 1 activity, leading to PKC-dependent insulin exocytosis. Am J Physiol Endocrinol Metab. 2003 Aug;285(2):E438-46. Epub 2003 Apr 8. [PubMed:12684219]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Xanthine oxidase activity
Specific Function
Key enzyme in purine degradation. Catalyzes the oxidation of hypoxanthine to xanthine. Catalyzes the oxidation of xanthine to uric acid. Contributes to the generation of reactive oxygen species. Ha...
Gene Name
XDH
Uniprot ID
P47989
Uniprot Name
Xanthine dehydrogenase/oxidase
Molecular Weight
146422.99 Da
References
  1. Di Giacomo C, Latteri F, Fichera C, Sorrenti V, Campisi A, Castorina C, Russo A, Pinturo R, Vanella A: Effect of acetyl-L-carnitine on lipid peroxidation and xanthine oxidase activity in rat skeletal muscle. Neurochem Res. 1993 Nov;18(11):1157-62. [PubMed:8255367]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Triglyceride lipase activity
Specific Function
Involved in the detoxification of xenobiotics and in the activation of ester and amide prodrugs. Hydrolyzes aromatic and aliphatic esters, but has no catalytic activity toward amides or a fatty acy...
Gene Name
CES1
Uniprot ID
P23141
Uniprot Name
Liver carboxylesterase 1
Molecular Weight
62520.62 Da
References
  1. Bell FP: Carnitine ester hydrolysis in arteries from normal and cholesterol-fed rabbits and the effects of carnitine esters on arterial microsomal ACAT. Comp Biochem Physiol B. 1984;79(2):125-8. [PubMed:6509906]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
General Function
Peroxidase activity
Specific Function
Part of the host defense system of polymorphonuclear leukocytes. It is responsible for microbicidal activity against a wide range of organisms. In the stimulated PMN, MPO catalyzes the production o...
Gene Name
MPO
Uniprot ID
P05164
Uniprot Name
Myeloperoxidase
Molecular Weight
83867.71 Da
References
  1. Derin N, Agac A, Bayram Z, Asar M, Izgut-Uysal VN: Effects of L-carnitine on neutrophil-mediated ischemia-reperfusion injury in rat stomach. Cell Biochem Funct. 2006 Sep-Oct;24(5):437-42. [PubMed:16130180]

Transporters

Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Substrate
Inhibitor
General Function
Symporter activity
Specific Function
Sodium-ion dependent, low affinity carnitine transporter. Probably transports one sodium ion with one molecule of carnitine. Also transports organic cations such as tetraethylammonium (TEA) without...
Gene Name
SLC22A4
Uniprot ID
Q9H015
Uniprot Name
Solute carrier family 22 member 4
Molecular Weight
62154.48 Da
References
  1. Yabuuchi H, Tamai I, Nezu J, Sakamoto K, Oku A, Shimane M, Sai Y, Tsuji A: Novel membrane transporter OCTN1 mediates multispecific, bidirectional, and pH-dependent transport of organic cations. J Pharmacol Exp Ther. 1999 May;289(2):768-73. [PubMed:10215651]
  2. Tamai I, Ohashi R, Nezu JI, Sai Y, Kobayashi D, Oku A, Shimane M, Tsuji A: Molecular and functional characterization of organic cation/carnitine transporter family in mice. J Biol Chem. 2000 Dec 22;275(51):40064-72. [PubMed:11010964]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Inhibitor
General Function
Sodium-independent organic anion transmembrane transporter activity
Specific Function
Mediates the Na(+)-independent uptake of organic anions such as pravastatin, taurocholate, methotrexate, dehydroepiandrosterone sulfate, 17-beta-glucuronosyl estradiol, estrone sulfate, prostagland...
Gene Name
SLCO1B1
Uniprot ID
Q9Y6L6
Uniprot Name
Solute carrier organic anion transporter family member 1B1
Molecular Weight
76447.99 Da
References
  1. Nozawa T, Tamai I, Sai Y, Nezu J, Tsuji A: Contribution of organic anion transporting polypeptide OATP-C to hepatic elimination of the opioid pentapeptide analogue [D-Ala2, D-Leu5]-enkephalin. J Pharm Pharmacol. 2003 Jul;55(7):1013-20. [PubMed:12906759]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Substrate
General Function
Symporter activity
Specific Function
Sodium-ion dependent, high affinity carnitine transporter. Involved in the active cellular uptake of carnitine. Transports one sodium ion with one molecule of carnitine. Also transports organic cat...
Gene Name
SLC22A5
Uniprot ID
O76082
Uniprot Name
Solute carrier family 22 member 5
Molecular Weight
62751.08 Da
References
  1. 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]
  2. Friedrich A, Prasad PD, Freyer D, Ganapathy V, Brust P: Molecular cloning and functional characterization of the OCTN2 transporter at the RBE4 cells, an in vitro model of the blood-brain barrier. Brain Res. 2003 Apr 4;968(1):69-79. [PubMed:12644265]
  3. Elimrani I, Lahjouji K, Seidman E, Roy MJ, Mitchell GA, Qureshi I: Expression and localization of organic cation/carnitine transporter OCTN2 in Caco-2 cells. Am J Physiol Gastrointest Liver Physiol. 2003 May;284(5):G863-71. Epub 2003 Jan 10. [PubMed:12684216]
  4. Ohashi R, Tamai I, Yabuuchi H, Nezu JI, Oku A, Sai Y, Shimane M, Tsuji A: Na(+)-dependent carnitine transport by organic cation transporter (OCTN2): its pharmacological and toxicological relevance. J Pharmacol Exp Ther. 1999 Nov;291(2):778-84. [PubMed:10525100]
  5. Tamai I, Ohashi R, Nezu JI, Sai Y, Kobayashi D, Oku A, Shimane M, Tsuji A: Molecular and functional characterization of organic cation/carnitine transporter family in mice. J Biol Chem. 2000 Dec 22;275(51):40064-72. [PubMed:11010964]
  6. Wu X, Huang W, Prasad PD, Seth P, Rajan DP, Leibach FH, Chen J, Conway SJ, Ganapathy V: Functional characteristics and tissue distribution pattern of organic cation transporter 2 (OCTN2), an organic cation/carnitine transporter. J Pharmacol Exp Ther. 1999 Sep;290(3):1482-92. [PubMed:10454528]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Substrate
General Function
Organic cation transmembrane transporter activity
Specific Function
High affinity carnitine transporter; the uptake is partially sodium-ion dependent. Thought to mediate the L-carnitine secretion mechanism from testis epididymal epithelium into the lumen which is i...
Gene Name
SLC22A16
Uniprot ID
Q86VW1
Uniprot Name
Solute carrier family 22 member 16
Molecular Weight
64613.58 Da
References
  1. Enomoto A, Wempe MF, Tsuchida H, Shin HJ, Cha SH, Anzai N, Goto A, Sakamoto A, Niwa T, Kanai Y, Anders MW, Endou H: Molecular identification of a novel carnitine transporter specific to human testis. Insights into the mechanism of carnitine recognition. J Biol Chem. 2002 Sep 27;277(39):36262-71. Epub 2002 Jun 27. [PubMed:12089149]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Substrate
General Function
Sodium-independent organic anion transmembrane transporter activity
Specific Function
Plays an important role in the excretion/detoxification of endogenous and exogenous organic anions, especially from the brain and kidney. Involved in the transport basolateral of steviol, fexofenad...
Gene Name
SLC22A8
Uniprot ID
Q8TCC7
Uniprot Name
Solute carrier family 22 member 8
Molecular Weight
59855.585 Da
References
  1. Kobayashi Y, Ohshiro N, Tsuchiya A, Kohyama N, Ohbayashi M, Yamamoto T: Renal transport of organic compounds mediated by mouse organic anion transporter 3 (mOat3): further substrate specificity of mOat3. Drug Metab Dispos. 2004 May;32(5):479-83. [PubMed:15100168]

Drug created on June 13, 2005 07:24 / Updated on October 21, 2017 09:34