The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
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Protein Aliases: Carnitine O-palmitoyltransferase 1, liver isoform; Carnitine O-palmitoyltransferase I, liver isoform; Carnitine palmitoyltransferase 1A; carnitine palmitoyltransferase 1A (liver); carnitine palmitoyltransferase I; carnitine palmitoyltransferase I, liver; CPT I; CPT-1; CPT1; CPT1-L; CPT1A; CPTI-L; L-CPT1
Gene Aliases: CPT1; CPT1-L; CPT1A; L-CPT1
UniProt ID: (Human) P50416
Entrez Gene ID: (Human) 1374