Carnitine palmitoyltransferase 1C (CPT1C)

Carnitine palmitoyltransferase 1C (CPT1C) is a mammalian-specific member of the carnitine palmitoyltransferase 1 enzyme family, primarily expressed in the central nervous system (neurons), some stem cells, and certain cancer cells. Unlike the classical CPT1A and CPT1B isoforms, which localize to the mitochondrial outer membrane and catalyze the transfer of long-chain fatty acids into mitochondria for β-oxidation, CPT1C is mainly found in the endoplasmic reticulum and functions primarily as a nutrient sensor with very low acyltransferase activity. CPT1C binds malonyl-CoA and modulates the activity of interaction partners involved in lipid metabolism, secretory transport, Golgi and endolysosomal pathways, thereby impacting neuronal energy homeostasis, axonal outgrowth, and cellular stress adaptation. Mutations in CPT1C cause hereditary spastic paraplegia (SPG73) and CPT1C expression serves as a marker of metabolic adaptation and poor prognosis in various tumors. No specific drugs directly targeting CPT1C are currently in clinical use; its function and interactions are sensitive chiefly to metabolic intermediates such as malonyl-CoA.

Other names

Palmitoyl thioesterase CPT1CCATL1CPTI-BCPT I-CFLJ23809CPTICCPT1PCarnitine O-palmitoyltransferase 1, brain isoformCarnitine palmitoyltransferase I (brain isoform)Carnitine palmitoyltransferase I related CSPG73

Nutritional/metabolic sensing via malonyl-CoA binding, modulating protein-protein interactions and cellular metabolic adaptation