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Our body produces L-carnitine from the essential amino acid lysine via a specific biosynthetic pathway. Healthy individuals, including strict vegetarians, generally synthesize enough L-carnitine to prevent deficiency. However, certain conditions like pregnancy may result in increased excretion of L-carnitine, potentially increasing the risk for deficiency. (More information)
Because of its role in the transport of long-chain fatty acids from the cytosol to the mitochondrial matrix, L-carnitine is critical for mitochondrial fatty acid β-oxidation. (More information)
L-Carnitine supplementation is indicated for the treatment of primary systemic carnitine deficiency, which is caused by mutations in the gene that codes for the carnitine transporter, OCTN2. (More information)
L-Carnitine is also approved for the treatment of carnitine deficiencies secondary to inherited diseases, such as propionyl-CoA carboxylase deficiency and medium chain acyl-CoA dehydrogenase deficiency, and in patients with end-stage renal disease undergoing hemodialysis. (More information)
Evidence from randomized controlled trials suggests that L-carnitine or acylcarnitine esters may be useful adjuncts to standard medical treatment in individuals with cardiovascular disease. (More information)
Routine administration of L-carnitine to people with end-stage renal disease undergoing hemodialysis is not recommended unless it is to treat carnitine deficiency.