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Chemical Compound Review

Carnitine     3-hydroxy-4-trimethylammonio- butanoate

Synonyms: Excitine, Miotonal, Vitacarn, Carnitina, Novaine, ...
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Disease relevance of Carnitine


Psychiatry related information on Carnitine


High impact information on Carnitine

  • Carnitine is synthesized in most eucaryotic organisms, although a few insects (and most likely some newborn animals) require it as a nutritional factor (vitamin BT) [11].
  • The half-life of plasma carnitine for cystinotic children given a single oral dose approximated 6.3 h; 14% of ingested L-carnitine was excreted within 24 h [12].
  • We suggest that L-carnitine may be a useful therapeutic approach for elimination of toxic acyl CoA compounds in several of these disorders [13].
  • Our results also suggested that a calmodulin- but not a protein kinase C- or protein kinase A-mediated pathway plays a role in regulating L-carnitine uptake by Caco-2 cells [14].
  • CONCLUSIONS: L-carnitine uptake by intestinal epithelial cells (Caco-2) involves a carrier-mediated system that is temperature-, Na(+)-, and energy-dependent and seems to be under the regulation of a calmodulin-mediated pathway [14].

Chemical compound and disease context of Carnitine


Biological context of Carnitine


Anatomical context of Carnitine


Associations of Carnitine with other chemical compounds


Gene context of Carnitine

  • Here, we report the first detailed functional characterization of the caiT gene product, a putative transport protein whose function is required for l-carnitine conversion in Escherichia coli [15].
  • The gene YAT1 from Saccharomyces cerevisiae encodes a protein of 688 amino acids which displays significant sequence similarity to vertebrate L-carnitine acyltransferases and yeast inner mitochondrial L-carnitine acetyltransferase [31].
  • This impairment of l-carnitine transport by OCTN1 may respond to high-dose l-carnitine therapy [32].
  • The results showed that, except from infertility, L-carnitine treatment resulted in a significant increase of macrophages and to a lesser degree an increase of T-cells, while elevated levels of IFN-gamma and TNF-alpha were detected in both serum and peritoneal fluid compared to controls [33].
  • Under these conditions, an E. coli K-12 strain with lesions in both proP and proU accumulates low levels of L-carnitine but fails to accumulate betaine when these compounds are supplied in the external medium [34].

Analytical, diagnostic and therapeutic context of Carnitine


  1. L-carnitine for treatment of distal ulcerative colitis. Johnson, J.A. Gastroenterology (1992) [Pubmed]
  2. L-carnitine for leg ulcers. Harrell, H.L. Ann. Intern. Med. (1990) [Pubmed]
  3. Increases in walking distance in patients with peripheral vascular disease treated with L-carnitine: a double-blind, cross-over study. Brevetti, G., Chiariello, M., Ferulano, G., Policicchio, A., Nevola, E., Rossini, A., Attisano, T., Ambrosio, G., Siliprandi, N., Angelini, C. Circulation (1988) [Pubmed]
  4. Pivotal role of Harakiri in the induction and prevention of gentamicin-induced hearing loss. Kalinec, G.M., Fernandez-Zapico, M.E., Urrutia, R., Esteban-Cruciani, N., Chen, S., Kalinec, F. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  5. Effect of L-carnitine on human immunodeficiency virus-1 infection-associated apoptosis: a pilot study. Moretti, S., Alesse, E., Di Marzio, L., Zazzeroni, F., Ruggeri, B., Marcellini, S., Famularo, G., Steinberg, S.M., Boschini, A., Cifone, M.G., De Simone, C. Blood (1998) [Pubmed]
  6. Superiority of L-propionylcarnitine vs L-carnitine in improving walking capacity in patients with peripheral vascular disease: an acute, intravenous, double-blind, cross-over study. Brevetti, G., Perna, S., Sabbà, C., Rossini, A., Scotto di Uccio, V., Berardi, E., Godi, L. Eur. Heart J. (1992) [Pubmed]
  7. Double-blind parallel design pilot study of acetyl levocarnitine in patients with Alzheimer's disease. Sano, M., Bell, K., Cote, L., Dooneief, G., Lawton, A., Legler, L., Marder, K., Naini, A., Stern, Y., Mayeux, R. Arch. Neurol. (1992) [Pubmed]
  8. L-carnitine suppresses the onset of neuromuscular degeneration and increases the life span of mice with familial amyotrophic lateral sclerosis. Kira, Y., Nishikawa, M., Ochi, A., Sato, E., Inoue, M. Brain Res. (2006) [Pubmed]
  9. Suppression of pentylenetetrazol-induced seizures by carnitine in mice. Yu, Z., Iryo, Y., Matsuoka, M., Igisu, H., Ikeda, M. Naunyn Schmiedebergs Arch. Pharmacol. (1997) [Pubmed]
  10. Medium-term open label trial of L-carnitine in Rett syndrome. Ellaway, C.J., Peat, J., Williams, K., Leonard, H., Christodoulou, J. Brain Dev. (2001) [Pubmed]
  11. Carnitine--metabolism and functions. Bremer, J. Physiol. Rev. (1983) [Pubmed]
  12. Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome. Gahl, W.A., Bernardini, I., Dalakas, M., Rizzo, W.B., Harper, G.S., Hoeg, J.M., Hurko, O., Bernar, J. J. Clin. Invest. (1988) [Pubmed]
  13. L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia. Roe, C.R., Millington, D.S., Maltby, D.A., Bohan, T.P., Hoppel, C.L. J. Clin. Invest. (1984) [Pubmed]
  14. Uptake of L-carnitine by a human intestinal epithelial cell line, Caco-2. McCloud, E., Ma, T.Y., Grant, K.E., Mathis, R.K., Said, H.M. Gastroenterology (1996) [Pubmed]
  15. CaiT of Escherichia coli, a new transporter catalyzing L-carnitine/gamma -butyrobetaine exchange. Jung, H., Buchholz, M., Clausen, J., Nietschke, M., Revermann, A., Schmid, R., Jung, K. J. Biol. Chem. (2002) [Pubmed]
  16. Effect of L-carnitine treatment on lipid metabolism and cardiac performance in chronically diabetic rats. Rodrigues, B., Xiang, H., McNeill, J.H. Diabetes (1988) [Pubmed]
  17. Synthesis and biological evaluation of cyclic analogues of 1-carnitine as potential agents in the treatment of myocardial ischemia. Woster, P.M., Murray, W.J. J. Med. Chem. (1986) [Pubmed]
  18. Effects of L-carnitine and verapamil on myocardial carnitine concentration and histopathology of Syrian hamster BIO 14.6. Yamashita, T., Kobayashi, A., Yamazaki, N., Miura, K., Shirasawa, H. Cardiovasc. Res. (1986) [Pubmed]
  19. Impact of mitochondrial beta-oxidation in fatty acid-mediated inhibition of glioma cell proliferation. Berge, K., Tronstad, K.J., Bohov, P., Madsen, L., Berge, R.K. J. Lipid Res. (2003) [Pubmed]
  20. Inhibition by palmitoylcarnitine of adhesion and morphological changes in HL-60 cells induced by 12-O-tetradecanoylphorbol-13-acetate. Nakaki, T., Mita, S., Yamamoto, S., Nakadate, T., Kato, R. Cancer Res. (1984) [Pubmed]
  21. Loss of wild-type carrier-mediated L-carnitine transport activity in hepatocytes of juvenile visceral steatosis mice. Yokogawa, K., Yonekawa, M., Tamai, I., Ohashi, R., Tatsumi, Y., Higashi, Y., Nomura, M., Hashimoto, N., Nikaido, H., Hayakawa, J., Nezu, J., Oku, A., Shimane, M., Miyamoto, K., Tsuji, A. Hepatology (1999) [Pubmed]
  22. Sigmoid kinetics of purified beef heart mitochondrial carnitine palmitoyltransferase. Effect of pH and malonyl-CoA. Fiol, C.J., Bieber, L.L. J. Biol. Chem. (1984) [Pubmed]
  23. Carnitine transport in human intestinal biopsy specimens. Demonstration of an active transport system. Hamilton, J.W., Li, B.U., Shug, A.L., Olsen, W.A. Gastroenterology (1986) [Pubmed]
  24. A genome-wide screen in Saccharomyces cerevisiae reveals altered transport as a mechanism of resistance to the anticancer drug bleomycin. Aouida, M., Pagé, N., Leduc, A., Peter, M., Ramotar, D. Cancer Res. (2004) [Pubmed]
  25. L-carnitine prevents doxorubicin-induced apoptosis of cardiac myocytes: role of inhibition of ceramide generation. Andrieu-Abadie, N., Jaffrezou, J.P., Hatem, S., Laurent, G., Levade, T., Mercadier, J.J. FASEB J. (1999) [Pubmed]
  26. Carnitine acetyltransferase activity in the human brain and its microvessels is decreased in Alzheimer's disease. Kalaria, R.N., Harik, S.I. Ann. Neurol. (1992) [Pubmed]
  27. Carnitine stimulation of glucose oxidation in the fatty acid perfused isolated working rat heart. Broderick, T.L., Quinney, H.A., Lopaschuk, G.D. J. Biol. Chem. (1992) [Pubmed]
  28. Mitochondrial metabolism of pyruvate in bovine spermatozoa. Hutson, S.M., Van Dop, C., Lardy, H.A. J. Biol. Chem. (1977) [Pubmed]
  29. Peroxisomal beta-oxidation of branched chain fatty acids in rat liver. Evidence that carnitine palmitoyltransferase I prevents transport of branched chain fatty acids into mitochondria. Singh, H., Beckman, K., Poulos, A. J. Biol. Chem. (1994) [Pubmed]
  30. Redesign of choline acetyltransferase specificity by protein engineering. Cronin, C.N. J. Biol. Chem. (1998) [Pubmed]
  31. The ethanol-inducible YAT1 gene from yeast encodes a presumptive mitochondrial outer carnitine acetyltransferase. Schmalix, W., Bandlow, W. J. Biol. Chem. (1993) [Pubmed]
  32. Epitope shared by functional variant of organic cation/carnitine transporter, OCTN1, Campylobacter jejuni and Mycobacterium paratuberculosis may underlie susceptibility to Crohn's disease at 5q31. Lamhonwah, A.M., Ackerley, C., Onizuka, R., Tilups, A., Lamhonwah, D., Chung, C., Tao, K.S., Tellier, R., Tein, I. Biochem. Biophys. Res. Commun. (2005) [Pubmed]
  33. Constitutive or induced elevated levels of L-carnitine correlate with the cytokine and cellular profile of endometriosis. Dionyssopoulou, E., Vassiliadis, S., Evangeliou, A., Koumantakis, E.E., Athanassakis, I. J. Reprod. Immunol. (2005) [Pubmed]
  34. A possible role of ProP, ProU and CaiT in osmoprotection of Escherichia coli by carnitine. Verheul, A., Wouters, J.A., Rombouts, F.M., Abee, T. J. Appl. Microbiol. (1998) [Pubmed]
  35. L-carnitine therapy in isovaleric acidemia. Roe, C.R., Millington, D.S., Maltby, D.A., Kahler, S.G., Bohan, T.P. J. Clin. Invest. (1984) [Pubmed]
  36. Effects of L-carnitine supplementation in maintenance hemodialysis patients: a systematic review. Hurot, J.M., Cucherat, M., Haugh, M., Fouque, D. J. Am. Soc. Nephrol. (2002) [Pubmed]
  37. L-carnitine: effect of intravenous administration on fuel homeostasis in normal subjects and home-parenteral-nutrition patients with low plasma carnitine concentrations. Bowyer, B.A., Fleming, C.R., Haymond, M.W., Miles, J.M. Am. J. Clin. Nutr. (1989) [Pubmed]
  38. Pharmacokinetics of L-carnitine in patients with end-stage renal disease undergoing long-term hemodialysis. Evans, A.M., Faull, R., Fornasini, G., Lemanowicz, E.F., Longo, A., Pace, S., Nation, R.L. Clin. Pharmacol. Ther. (2000) [Pubmed]
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