| 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-oxidation in the mitochondrion. Possesses also a lysine succinyltransferase activity that can regulate enzymatic activity of substrate proteins such as ENO1 and metabolism independent of its classical carnitine O-palmitoyltransferase activity. Plays an important role in hepatic triglyceride metabolism. Plays also a role in inducible regulatory T-cell (iTreg) differentiation once activated by butyryl-CoA that antagonizes malonyl-CoA-mediated CPT1A repression. Sustains the IFN-I response by recruiting ZDHCC4 to palmitoylate MAVS at the mitochondria leading to MAVS stabilization and activation. Promotes ROS-induced oxidative stress in liver injury via modulation of NFE2L2 and NLRP3-mediated signaling pathways. |
| Protein Name | Carnitine O-Palmitoyltransferase 1 - Liver IsoformCpt1-LCarnitine O-Palmitoyltransferase I - Liver IsoformCpt ICpti-LCarnitine Palmitoyltransferase 1aSuccinyltransferase Cpt1a |
| Database Links | Reactome: R-HSA-1368082Reactome: R-HSA-1989781Reactome: R-HSA-200425Reactome: R-HSA-5362517 |
| Cellular Localisation | Mitochondrion Outer MembraneMulti-Pass Membrane Protein |
| Alternative Protein Names | Carnitine O-Palmitoyltransferase 1 - Liver Isoform proteinCpt1-L proteinCarnitine O-Palmitoyltransferase I - Liver Isoform proteinCpt I proteinCpti-L proteinCarnitine Palmitoyltransferase 1a proteinSuccinyltransferase Cpt1a proteinCPT1A proteinCPT1 protein |
Information sourced from Uniprot.org
