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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: Carnitine O-palmitoyltransferase 1, liver isoform; Carnitine O-palmitoyltransferase I, liver isoform; carnitine palmitoyltransferase 1, liver; Carnitine palmitoyltransferase 1A; carnitine palmitoyltransferase 1A (liver); carnitine palmitoyltransferase I, liver; CPT I; CPT1-L; CPTI-L; L-CPT I
Gene Aliases: C730027G07; Cpt-1; CPT1; CPT1-L; CPT1A; CPTI; L-CPT1