Target type: molecularfunction
Catalysis of the reaction: palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine. [EC:2.3.1.21]
Carnitine O-palmitoyltransferase (CPT) activity is a critical step in the beta-oxidation pathway, responsible for the breakdown of long-chain fatty acids for energy production. The enzyme catalyzes the transfer of long-chain fatty acyl groups from coenzyme A (CoA) to carnitine, forming acylcarnitine. This reaction is crucial for transporting long-chain fatty acids across the mitochondrial membrane.
The process begins with the activation of fatty acids to their CoA derivatives by acyl-CoA synthetase. This activated fatty acyl-CoA then interacts with CPT, which is located in the outer mitochondrial membrane. CPT catalyzes the transfer of the fatty acyl group from CoA to carnitine, generating acylcarnitine. Acylcarnitine is then transported into the mitochondrial matrix by the carnitine acylcarnitine translocase (CACT).
Once inside the mitochondria, the acylcarnitine is hydrolyzed by carnitine palmitoyltransferase II (CPT II), releasing carnitine and the free fatty acyl-CoA. This free fatty acyl-CoA then enters the beta-oxidation pathway, undergoing a series of reactions that generate energy in the form of ATP.
In summary, carnitine O-palmitoyltransferase activity facilitates the transport of long-chain fatty acids from the cytoplasm into the mitochondria, where they can be oxidized for energy production. This enzyme plays a critical role in energy metabolism, particularly during periods of fasting, exercise, or dietary fat intake.'
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Protein | Definition | Taxonomy |
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Carnitine O-palmitoyltransferase 1, muscle isoform | A 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 isoform | A 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, mitochondrial | A carnitine O-palmitoyltransferase 2, mitochondrial that is encoded in the genome of human. [PRO:DNx, UniProtKB:P23786] | Homo sapiens (human) |
Carnitine O-palmitoyltransferase 1, muscle isoform | A 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 isoform | A 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, mitochondrial | A carnitine O-palmitoyltransferase 2, mitochondrial that is encoded in the genome of human. [PRO:DNx, UniProtKB:P23786] | Homo sapiens (human) |
Compound | Definition | Classes | Roles |
---|---|---|---|
4-hydroxyphenylglyoxylic acid | 4-hydroxyphenylglyoxylate : Conjugate base of 4-hydroxyphenylglyoxylic acid. 4-hydroxyphenylglyoxylic acid: RN given refers to parent cpd | phenols | |
amiodarone | amiodarone : 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 |
perhexiline | Perhexiline: 2-(2,2-Dicyclohexylethyl)piperidine. Coronary vasodilator used especially for angina of effort. It may cause neuropathy and hepatitis. | piperidines | cardiovascular drug |
pioglitazone | pioglitazone : 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 |
cordycepin | 3'-deoxyribonucleoside; adenosines | antimetabolite; nucleoside antibiotic | |
trimetazidine | Trimetazidine: A vasodilator used in angina of effort or ischemic heart disease. | aromatic amine | |
4-hydroxyphenylglycine, (s)-isomer | L-4-hydroxyphenylglycine : The L-enantiomer of 4-hydroxyphenylglycine. | 4-hydroxyphenylglycine | |
rosiglitazone | aminopyridine; thiazolidinediones | EC 6.2.1.3 (long-chain-fatty-acid--CoA ligase) inhibitor; ferroptosis inhibitor; insulin-sensitizing drug | |
tebuconazole | 1-(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 | |
corydaline | isoquinoline alkaloid; isoquinolines | ||
emeriamine | emeriamine: 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]acetamide | organic 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-oxadiazole | benzodioxoles | ||
4-trimethylammonio-3-((tetradecylcarbamoyl)amino)butyrate | |||
formycin b | formycin B: RN given refers to (beta-D)-isomer | formycin |