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carnitine metabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways involving carnitine (hydroxy-trimethyl aminobutyric acid), a compound that participates in the transfer of acyl groups across the inner mitochondrial membrane. [GOC:jl, ISBN:0198506732]

Carnitine metabolism is a crucial process for energy production, particularly in tissues with high energy demands like the heart and skeletal muscles. It involves the transport of long-chain fatty acids (LCFAs) across the mitochondrial membrane, where they are broken down through beta-oxidation to generate ATP. This process begins with the synthesis of L-carnitine from lysine and methionine. L-carnitine is then transported into the mitochondria via carnitine palmitoyltransferase I (CPT1) and carnitine palmitoyltransferase II (CPT2), which catalyze the reversible transfer of LCFAs from coenzyme A (CoA) to L-carnitine. The resulting acylcarnitine is transported into the mitochondrial matrix where CPT2 releases the fatty acyl group. The fatty acyl-CoA is then subjected to beta-oxidation, a process that cleaves two-carbon units from the fatty acid chain, generating acetyl-CoA and reducing equivalents (NADH and FADH2) that drive ATP production through oxidative phosphorylation. The cycle is completed with the release of carnitine from the mitochondria and its recycling for further transport of LCFAs. Deficiencies in carnitine metabolism can lead to various metabolic disorders, affecting energy production and potentially causing muscle weakness, cardiomyopathy, and other health complications. '
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Proteins (4)

ProteinDefinitionTaxonomy
Solute carrier family 22 member 4A solute carrier family 22 member 4 that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q9H015]Homo sapiens (human)
Carnitine O-palmitoyltransferase 1, muscle isoformA carnitine O-palmitoyltransferase 1, muscle isoform that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q92523]Homo sapiens (human)
Carnitine O-palmitoyltransferase 1, liver isoformA carnitine O-palmitoyltransferase 1, liver isoform that is encoded in the genome of human. [PRO:DNx, UniProtKB:P50416]Homo sapiens (human)
Carnitine O-palmitoyltransferase 2, mitochondrialA carnitine O-palmitoyltransferase 2, mitochondrial that is encoded in the genome of human. [PRO:DNx, UniProtKB:P23786]Homo sapiens (human)

Compounds (17)

CompoundDefinitionClassesRoles
4-hydroxyphenylglyoxylic acid4-hydroxyphenylglyoxylate : Conjugate base of 4-hydroxyphenylglyoxylic acid.

4-hydroxyphenylglyoxylic acid: RN given refers to parent cpd
phenols
amiodaroneamiodarone : A member of the class of 1-benzofurans that is 1-benzofuran substituted by a butyl group at position 2 and a 4-[2-(diethylamino)ethoxy]-3,5-diiodobenzoyl group at position 3. It is a cardiovascular drug used for the treatment of cardiac dysrhythmias.

Amiodarone: An antianginal and class III antiarrhythmic drug. It increases the duration of ventricular and atrial muscle action by inhibiting POTASSIUM CHANNELS and VOLTAGE-GATED SODIUM CHANNELS. There is a resulting decrease in heart rate and in vascular resistance.
1-benzofurans;
aromatic ketone;
organoiodine compound;
tertiary amino compound
cardiovascular drug
perhexilinePerhexiline: 2-(2,2-Dicyclohexylethyl)piperidine. Coronary vasodilator used especially for angina of effort. It may cause neuropathy and hepatitis.piperidinescardiovascular drug
pioglitazonepioglitazone : A member of the class of thiazolidenediones that is 1,3-thiazolidine-2,4-dione substituted by a benzyl group at position 5 which in turn is substituted by a 2-(5-ethylpyridin-2-yl)ethoxy group at position 4 of the phenyl ring. It exhibits hypoglycemic activity.

Pioglitazone: A thiazolidinedione and PPAR GAMMA agonist that is used in the treatment of TYPE 2 DIABETES MELLITUS.
aromatic ether;
pyridines;
thiazolidinediones
antidepressant;
cardioprotective agent;
EC 2.7.1.33 (pantothenate kinase) inhibitor;
EC 6.2.1.3 (long-chain-fatty-acid--CoA ligase) inhibitor;
ferroptosis inhibitor;
geroprotector;
hypoglycemic agent;
insulin-sensitizing drug;
PPARgamma agonist;
xenobiotic
tetraethylammoniumTetraethylammonium: A potassium-selective ion channel blocker. (From J Gen Phys 1994;104(1):173-90)quaternary ammonium ion
cordycepin3'-deoxyribonucleoside;
adenosines
antimetabolite;
nucleoside antibiotic
trimetazidineTrimetazidine: A vasodilator used in angina of effort or ischemic heart disease.aromatic amine
4-hydroxyphenylglycine, (s)-isomerL-4-hydroxyphenylglycine : The L-enantiomer of 4-hydroxyphenylglycine.4-hydroxyphenylglycine
rosiglitazoneaminopyridine;
thiazolidinediones
EC 6.2.1.3 (long-chain-fatty-acid--CoA ligase) inhibitor;
ferroptosis inhibitor;
insulin-sensitizing drug
tebuconazole1-(4-chlorophenyl)-4,4-dimethyl-3-(1H-1,2,4-triazol-1-ylmethyl)pentan-3-ol : A tertiary alcohol that is pentan-3-ol substituted by a 4-chlorophenyl, methyl, methyl, and a 1H-1,2,4-triazol-1-ylmethyl at positions 1, 4, 4 and 3 respectively.

Lynx: A genus in the family FELIDAE comprising felines with long legs, ear tufts, and a short tail.

tebuconazole : A racemate composed of equimolar amounts of (R)- and (S)-tebuconazole. A fungicide effective against various smut and bunt diseases in cereals and other field crops.
monochlorobenzenes;
tertiary alcohol;
triazoles
corydalineisoquinoline alkaloid;
isoquinolines
emeriamineemeriamine: derived from fungal metabolite emericedin; structure given in first source
N-(1,3-benzodioxol-5-yl)-2-[[3-(2-methoxyphenyl)-4-oxo-6,7-dihydrothieno[3,2-d]pyrimidin-2-yl]thio]acetamideorganic heterobicyclic compound;
organonitrogen heterocyclic compound;
organosulfur heterocyclic compound
2-(1,3-benzodioxol-5-yl)-5-[[2-(4-methoxyphenyl)-4,5-dihydrothiazol-5-yl]methylthio]-1,3,4-oxadiazolebenzodioxoles
ergothioneineergothioneine thione form : A L-histidine derivative that is N(alpha),N(alpha),N(alpha)-trimethyl-L-histidine in which the hydrogen at position 2 on the imdazole ring is replaced by a thioxo group.1,3-dihydroimidazole-2-thiones;
amino-acid betaine;
L-histidine derivative;
sulfur-containing amino acid
antioxidant;
chelator;
fungal metabolite;
plant metabolite;
xenobiotic metabolite
4-trimethylammonio-3-((tetradecylcarbamoyl)amino)butyrate
formycin bformycin B: RN given refers to (beta-D)-isomerformycin