|Other Names||Carnitine O-palmitoyltransferase 1, liver isoform, CPT1-L, Carnitine O-palmitoyltransferase I, liver isoform, CPT I, CPTI-L, Carnitine palmitoyltransferase 1A, CPT1A, CPT1|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism.|
|Cellular Location||Mitochondrion outer membrane; Multi- pass membrane protein|
|Tissue Location||Strong expression in kidney and heart, and lower in liver and skeletal muscle|
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Provided below are standard protocols that you may find useful for product applications.
The mitochondrial oxidation of long-chain fatty acids isinitiated by the sequential action of carnitinepalmitoyltransferase I (which is located in the outer membrane andis detergent-labile) and carnitine palmitoyltransferase II (whichis located in the inner membrane and is detergent-stable), togetherwith a carnitine-acylcarnitine translocase. CPT I is the key enzymein the carnitine-dependent transport across the mitochondrial innermembrane and its deficiency results in a decreased rate of fattyacid beta-oxidation. Alternatively spliced transcript variantsencoding different isoforms have been found for this gene.
Gessner, B.D., et al. Pediatrics 126(5):945-951(2010)Collins, S.A., et al. Mol. Genet. Metab. 101 (2-3), 200-204 (2010) :Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Song, S., et al. Mol. Cell. Endocrinol. 325 (1-2), 54-63 (2010) :Ruano, G., et al. Pharmacogenomics 11(7):959-971(2010)
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