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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.
|Gene Name:||carnitine palmitoyltransferase 1A (liver)|
|Synonyms:||CPT1A, CPT1, CPT1-L, CPT I, CPTI-L, L-CPT1|
|Target Sequences:||NM_001876 NP_001867.2 P50416|
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