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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.
C730027G07; carnitine O-palmitoyltransferase; carnitine O-palmitoyltransferase 1, liver isoform; Carnitine O-palmitoyltransferase I, liver isoform; carnitine palmitoyl transferase I isoform; Carnitine palmitoyltransferase 1 alpha, liver isoform; carnitine palmitoyltransferase 1, liver; carnitine palmitoyltransferase 1A; carnitine palmitoyltransferase 1A (liver); carnitine palmitoyltransferase 1a, liver; carnitine palmitoyltransferase I, liver; CPT 1; CPT I; Cpt1; Cpt-1; CPT1 L; CPT1A; CPT1-L; CPTI; CPT-Ia; CPTI-L; L-CPT I; L-CPT1; mitochondrial carnitine palmitoyltransferase 1A
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