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L-Carnitine-d9 is intended for use as an internal standard for the quantification of L-carnitine (Item No. 21489) by GC- or LC-MS. L-Carnitine is a conditionally essential nutrient.1 It is obtained from dietary sources or through the metabolism of lysine and methionine.2,3 L-Carnitine facilitates the transport of long-chain fatty acids into the mitochondrial matrix for β-oxidation, has other diverse roles on metabolism, and is involved in the maintenance of coenzyme A (CoA; Item No. 16147) stores. Plasma and/or tissue levels of L-carnitine are decreased in primary L-carnitine deficiency, a disorder characterized by impaired fatty acid oxidation, with symptoms varying depending on whether it is systemic or muscle-specific.1 Serum and tissue levels of L-carnitine are also reduced in secondary L-carnitine deficiencies caused by a variety of hereditary defects or acquired disorders.
WARNING This product is not for human or veterinary use.
1. L(–)-
2. Carnitine biosynthesis in mammals. Biochem. J. 361(Pt 3), 417-429 (2002).
3. Primary carnitine deficiency and cardiomyopathy. Korean. Circ. J. 43(12), 785-792 (2013).