L-camitine supplementation

Prolonged moderate-intensity exercise without and with L-camitine supplementation in patients with MCAD deficiency. 

Nishida, N., Sugimoto, T., Araki, A., Woo, M., Sakane, Y. Kobayashi, Y. (1981) Pediatr. Res. 22, 500-503. Carnitine metabolism in valproate-treated rats The effect of L-Camitine supplementation. Nagao, M., Parimoo, B. Tanaka, K. (1993) J. Biol Chem 268, 24114-24124. Developmental, nutritional, and hormonal regulation of tissue-specific expression of the genes encoding various acyl-CoA dehydrogenases and a-subunit of electron transfer flavoprotein in rat. 

Treatment often includes L-camitine supplementation for both disorders and hydroxocobalamin in MMA. 

Most patients with PROP and MMA are prescribed L-camitine supplements to prevent a secondary carnitine deficiency, and routine measurement of plasma or serum carnitine is recommended [5]. This analysis will include total carnitine, carnitine esters (esterified), and free carnitine concentrations. Low concentrations of free carnitine suggest a need to increase the supplementation dose. With supplementation, the total and ester fractions are often elevated. 

Nishida, R, Sugimoto, T, Araki, A., Woo, M., Sakane, Y. Kobayashi, Y. (1981) Pediatr. Res. 22, 500-503. Carnitine metabolism in valproate-treated rats The effect of L-Camitine supplementation. 

Colombani, R, Wenk, C, Kunz, I., ICrahenbuhl, S., Kuhnt, M., Arnold, M., Frey-Rindova, R, Frey, W., and Langhans, W., Effects of L-camitine supplementation on physical performance and energy metabolism of endurance trained athletes a doubleblind crossover study. Eur. J. Appl. Physiol., 73, 434-439, 1996. 

Birkenfeld, C., H. Kluge and K. Eder, 2006a L-camitine supplementation of sows during pregnancy improves the suckling behaviour of their offspring. Br. J. Nutr. 96, 334-342. 

Birkenfeld, C., A. Ramanau, H. Kluge, J. Spilke and K. Eder, 2005. Effect of dietary L-camitine supplementation on growth performance of piglets from control sows or sows treated with L-camitine during pregnancy and lactation. J. Anim. Physiol. Anim. Nutr. 89, 277-283. 

Doberenz, J., C. Birkenfeld, H. Kluge and K. Eder, 2006. Effects of l-camitine supplementation in pregnant sows on plasma concentrations of insulin-like growth factors, various hormones and metabolites and chorion characteristics. J. Anim. Physiol. Anim. Nutr. 90, 487-499. 

Ldsel, D., C. Kalbe and C. Rehfeldt, 2009. L-camitine supplementation during suckling intensifies the early postnatal skeletal myofiber formation in piglets of low birth weight J. Anim. Sci., 87, 2216-2226. 

Xi, L., K. Brown, J. Woodworth, K. Shim, B. Johnson and J. Odle, 2008. Maternal dietary L-camitine supplementation influences fetal carnitine status and stimulates carnitine palmitoyltransferase and pymvate dehydrogenase complex activities in swine. J. Nutr. 138, 2356-2362. 

Bachmann HU, Hoffmann A. Interaction of food supplement L-camitine with oral anticoagulant acenocoumarol. Swiss Med Wkly (2004) 134 385. 

L-camitine is given in many metabohc disorders as a supplement or to correct a carnitine deficiency. The dose of carnitine can vary between 50 and 100 mg/kg/day, and in some organic acidurias, as much as 200-300 mg/kg/24 days may be necessary, hi some of the long-chain fatty acid oxidation disorders, use of carnitine is controversial, and in the view of potential adverse effects (formation of car-diotoxic acylcamitines), supplementation at time of metabolic decompensation should be avoided [18]. 

Fries MH, et til. Isovaleric acidemia response to a leucine load after three weeks of supplementation with glycine, L-camitine, and combined glycine-carnitine therapy. J Pediatr. 1996 129(3) 449-52. 

Nutrition management of GA-1 consists of restricting lysine and tryptophan, supplementing L-camitine, and providing sufficient energy to prevent catabolism. 

Restrict Lysine and tryptophan Supplement L-camitine, riboflavin, ... 

L-carnitine supplementation is routinely provided to patients with GA-1 as a way to reduce intramitochondrial glutaryl-CoA and provide extracellular release without the synthesis of glutaric acid and 3-hydroxyglutaric acid. L-camitine conjugates with coenzyme A esters to form acylcarnitines. The typical L-carnitine dose is 75-100 mg/kg/day or sufficient quantities to maintain free L-camitine concentrations within the normal range [4], Large doses of enteral L-carnitine may cause loose stools or diarrhea [11], In the hospitalized patient with acute illness, a continuous infusion of intravenous L-carnitine is preferably provided. 

Restrict Propiogenic amino acids (valine, isoleucine, methionine, threonine) Supplement L-camitine Trial of intramuscular hydroxocobalamin in MMA ... 

Since L-camitine conjugates with toxic acyl-CoA metabolites produced in PROP and MM A, patients often develop a secondary carnitine deficiency. To prevent this, L-camitine in doses of 100-300 mg/ kg/day is prescribed on a routine basis [5, 11]. Some medical foods designed for PROP and MMA contain L-camitine, and this needs to be considered when determining the amount of supplement to prescribe. The IV form of L-camitine is often used during hospitalization for acute illness [3]. 

It is important to differentiate the two forms of carnitine, which are L-camitine and D-camitine (Dextro form). L-camitine is the physiologically active form and is endogenously produced within the human, whereas D-camitine is not physiologically active and is a synthetic. Research has shown that subjects given D-camitine saw a depletion of their endogenous stores of L-camitine, 3-which may manifest itself as carnitine deficiency, especially during intense bouts of exercise. Supplementation of D-camitine is therefore not recommended because it may have a deleterious effect on the body as well as performance. 

Siliprandi et al. supplemented with 2 g of carnitine before high-intensity exercise and found that PDH activity was stimulated and there was a reduction in both plasma lactate and pymvate. In a similar study, Vecchiet et al. administered L-camitine or a placebo 1 h before cycle ergometer exercise. The exercise was a... 

Karlic, H. and Lohninger, A., Supplementation of L-camitine in athletes does it make sense Nutrition, 20, 709-715, 2004. 

Toxicoiogy Eye, skin, and resp. tract irritant Precaution Avoid contact with moisture and strong oxidizing agents Hazardous Decomp. Prods. CO , NO , and HCI gas HMIS Health 2, Flammability 0, Reactivity 1 Uses Antioxidant In dietary supplements o-Acetyl-L-camitine hydrochloride. See Acetyl-L-camitine hydrochloride Acetylene carbinol. See Propargyl alcohol Acetylenic glycol. See Tetramethyl decynediol Acetyl ester of lanolin. See Acetylated lanolin N-Acetyl ethanolamine. See Acetamide MEA Achiote. See Annatto (Bbca orellana)... 

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