Carnitine Supplementation in Uremia

  • Peter Fürst
  • Anne Glöggler
  • Claudia Rössle
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 260)


In humans, L-carnitine (L- (-) -3-hydroxy-4-trimethylaminobutyrate, molecular weight 62 daltons) is synthesized in the liver, kidney and brain from the two essential amino acids lysine and methionine mediated by a multi-enzyme system1. Four co-factors — vitamin C, niacin, vitamin B6, and iron — are required by the various enzymes2 , 3 . The rare amino acid N-6-trimethyllysine (TML) is formed by posttranslational methylation of lysine residues with S-adenosylmethionine4. Once released by proteolytic action, it may serve as a precursor for the endogenous biosynthesis of carnitine5 . TML is converted to L-carnitine via four enzymatic steps (hydroxylation, aldol cleavage, oxidation and a second hydroxylation).


Hemodialysis Patient Free Carnitine Carnitine Deficiency Carnitine Level Carnitine Concentration 
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  1. 1.
    Rebouche, C.J.: Sites and regulation of carnitine biosynthesis in mammals. Fed. Proc. 41:2848 (1982).PubMedGoogle Scholar
  2. 2.
    Frenkel, R.A., and McGarry, J.D.: Carnitine biosynthesis, metabolism, and function. Academic Press, New York (1980).Google Scholar
  3. 3.
    Bremer, J.: Carnitine-metabolism and functions. Physiol. Rev. 63:1420 (1983).PubMedGoogle Scholar
  4. 4.
    Paik, W.K., Kim, S.: Protein methylation: chemical, enzymological, and biological significance. Advanc. Enzvmol. 42:227 (1975).Google Scholar
  5. 5.
    LaBadie, J., Dunn, W.A., and Aronson, N.N.: Hepatic synthesis of carnitine from protein-bound trimethyllysine. Lysosomal digestion of methyl-lysine-labeled asialo-fetuin. Biochem. J. 160:85 (1976).PubMedGoogle Scholar
  6. 6.
    Fritz, J.B.: Carnitine and its role in fatty acid metabolism. Adv. Lipid Res. 1:285 (1963).PubMedGoogle Scholar
  7. 7.
    McGarry, J.D.: New perspectives in the regulation of ketogenesis. Diabetes 28:517 (1979).PubMedGoogle Scholar
  8. 8.
    Borum, P.R.: Carnitine function. In: “Clinical Aspects of Human Carnitine Deficiency”, Borum, P.R., ed., Pergamon Press, New York (1986).Google Scholar
  9. 9.
    Guarnieri, G., Toigo, G., Crapesi, L., Situlin, R., Del Bianco, M.A., Corsi, M., LoGreco, P., Paviotti, G., Mioni, G., and Campanacci, L.: Carnitine metabolism in chronic renal failure. Kidney Int. 32 (suppl. 22):116 (1987) .Google Scholar
  10. 10.
    Bieber, L.L., and Lysiak, W.: Characteristics and functions of short-chain and medium-chain carnitine acyltransferases. In: “Clinical Aspects of Human Carnitine Deficiency”, Borum, P.R., ed., Pergamon Press, New York (1986).Google Scholar
  11. 11.
    Böhmer, T., Bergrem, H., and Eiklid, K.: Carnitine deficiency induced during intermittend haemodialysis for renal failure. Lancet i:126 (1978).CrossRefGoogle Scholar
  12. 12.
    Corsi, M. : Secondary carnitine deficiency in renal dialysis. In: “Clinical Aspects of Human Carnitine Deficiency”, Borum, P.R., ed., Plenum Press, New York (1986) .Google Scholar
  13. 13.
    Kohse, K.P., Graser, T.A., Rössle, C., Franz, H.E., and Fürst, P.: Effect of hemodialysis on plasma-free trimethyllysine. In: “Immune and Metabolic Aspects of Therapeutic Blood Purification Systems”. Proc. Int. Symp. Trondheim, Karger Basel (1986) .Google Scholar
  14. 14.
    Penn, D., and Schmidt-Sommerfeld, E.: Carnitine and carnitine esters in plasma and adipose tissue of chronic uremic patients undergoing hemodialysis. Metabolism 32:806 (1983).PubMedCrossRefGoogle Scholar
  15. 15.
    Wanner, C., Förstner-Wanner, S., Schaeffer, G., Schollmeyer, P., and Hörl, W.H.: Serum-free carnitine, carnitine esters and lipids in patients on peritoneal and hemodialysis. Am. J. Nephrol. 6:206 (1986).PubMedCrossRefGoogle Scholar
  16. 16.
    Rössle, C., Kohse, K.P., Kapp, W. , Franz, H.E., Glöggler, A., Bergström, J., and Fürst, P.: Disturbed carnitine metabolism in hemodialysis patients. Clin. Nutr. 5 (suppl.1):136 (1986).Google Scholar
  17. 17.
    Wanner, C., Förstner-Wanner, S., Rössle, C., Kohse, K.P., Fürst, P., Schollmeyer, P., and Hörl, W.H.: Carnitine metabolism in patients with chronic renal failure: effects of L-carnitine supplementation. Kidney Int. 32 (suppl. 22):132 (1987).Google Scholar
  18. 18.
    Guarnieri, G.F., Carretta, R., Toigo, G., and Campanacci, L.: Acetate intolerance in chronic uremic patients. Nephron 24:212 (1979).PubMedCrossRefGoogle Scholar
  19. 19.
    Ricanati, E.S., Tserng, K.Y., and Hoppel, C.L.: Abnormal fatty acid utilization during prolonged fasting in chronic uremia. Kidney Int. 32 (suppl. 22):145 (1987).Google Scholar
  20. 20.
    Vinay, P., Cardoso, M., Tejedor, A., Prud’homme, M., Levelille, M., Vinet, B., Courteau, M., Gongoux, A., Rengel, M., Capierre, L., and Piette, Y.: Acetate metabolism during hemodialysis: metabolic considerations. Am. J. Nephrol. 7:337 (1987).PubMedCrossRefGoogle Scholar
  21. 21.
    Engel, A.G., and Rebouche, C.J.: Carnitine metabolism and inborn errors. J. Inher. Metab. Dis. 7 (suppl. 1):38 (1984).PubMedCrossRefGoogle Scholar
  22. 22.
    Bertoli, M., Battistella, P.A., Vergani, L. , Naso, A., Gasparotto, M.L. , Romagnoli, G.F., and Angelini, C.: Carnitine deficiency induced during hemodialysis and hyperlipidemia: effect of replacement therapy. Am. J. Clin. Nutr. 34:1496 (1981).PubMedGoogle Scholar
  23. 23.
    Salvica, V., Bellinghieri, G., DiStefano, C., Corvaja, E., Consolo, F., Corsi, M., Maccari, F., Spagnoli, L.G., Villaschi, S., and Palmieri, G.: Plasma and muscle carnitine levels in hemodialysis patients with morphological-ultrastructural examination of muscle samples. Nephron 35:232 (1983).CrossRefGoogle Scholar
  24. 24.
    Moorthy, A.V., Rosenblum, M., Rajaram, R., and Shug, A.L.: A comparison of plasma and muscle carnitine levels in patients on peritoneal or hemodialysis for chronic renal failure. Am. J. Nephrol. 3:205 (1983).PubMedCrossRefGoogle Scholar
  25. 25.
    Mingardi, G., Bizzi, A., Cini, M., Licine, R., Mecca, G., and Garattini, S.: Carnitine balance in hemodialyzed patients. Clin. Nephrol. 13:269 (1980).PubMedGoogle Scholar
  26. 26.
    Fagher, B., Cederblad, G., Monti, M., Osson, L., Rasmussen, B., and Thysell, H.: Carnitine and left ventricular function in hemodialysis patients. Scand. J. Clin. Lab. Invest. 45:193 (1985) .PubMedCrossRefGoogle Scholar
  27. 28.
    Bazzi, C., DiDonato, S., Castaglione, A. , Bazzi, C., DiDonato, S., Castaglione, A., Corsi, M., and D’Amico, G.: Carnitine metabolism in short- and long-term maintenance hemodialysis. In: Clinical Aspects of Human Carnitine Deficiency, Borum, P.R., ed., Pergamon Press, New York (1986) .Google Scholar
  28. 29.
    Chan, M.K., Varghese, Z., and Moorhead, J.F.: Lipid abnormalities in uremia, dialysis and transplantation. Kidney Int. 19:625 (1981).PubMedCrossRefGoogle Scholar
  29. 30.
    Wanner, C., and Hörl, W.H.: Carnitine abnormalities in patients with renal insufficiency. Pathophysiological and therapeutical aspects. Nephron 50:89 (1988).PubMedCrossRefGoogle Scholar
  30. 31.
    Gerondaes, P., Alberti, K., and Agius, L.: Fatty acid metabolism in hepatocytes cultured with hypolipidaemic drugs. Biochem. J. 253:161 (1988).PubMedGoogle Scholar
  31. 32.
    Vacha, G.M., Giorcelli, G., Siliprandi, N., and Corsi, M.: Favourable effects of L-carnitine treatment on hypertriglyceridemia in hemodialysis patients: decisive role of low levels of high-density lipoproteincholesterol. Am. J. Clin. Nutr. 38:532 (1983).PubMedGoogle Scholar
  32. 33.
    Zilleruelo, G., Novak, M., Freundlich, M., Goldberg, R. , Abitol, C., and Strauss, J.: L-carnitine supplementation ameliorates hyperlipidemia in children on hemodialysis. In: “Clinical Aspects of Human Carnitine Deficiency”, Borum, P.R., ed., Pergamon Press, New York (1986).Google Scholar
  33. 34.
    Nilsson-Ehle, P., Cederblad, G., Fagher, B., Monti, M., and Thysell, H.: Plasma lipoproteins, liver function and glucose metabolism in hemodialysis patients: lack of effect of L-carnitine supplementation. Scand. J. Clin. Lab. Invest. 45:179 (1985).PubMedCrossRefGoogle Scholar
  34. 35.
    Ahmad, S., Golper, T., Hirschberg, R., Kopple, J., Katz, L., Kurtin, P., Ashbrook, D., and Wolfson, M.: Efficacy of L-carnitine in hemodialysis: a multi-center controlled clinical trial. Kidney Int. 31:226 (1987).Google Scholar
  35. 36.
    Yderstraede, K.B., Pedersen, F.B., Dragsholt, C., Trostmann, A., Laier, E., and Larsen, H.F.: The effect of L-carnitine on lipid-metabolism in patients on chronic hemodialysis. Nephrol. Dial. Transplant 1:238 (1987).PubMedGoogle Scholar
  36. 37.
    Böhles, H.J., and Akcetin, Z.: Ketogenic effects of low and high levels of carnitine during total parenteral nutrition in the rat. Am. J. Clin. Nutr. 46:47 (1987).PubMedGoogle Scholar
  37. 38.
    Guarnieri, G., Toigo, G., Situlin, R., DelBianco, M.A., Corsi, M., LoGreco, P., Paviotti, G., Mioni, G., and Campanacci, L.: Carnitine metabolism in chronic renal failure. Kidney Int. 32 (suppl. 22):116 (1987).Google Scholar
  38. 39.
    Glöggler, A., Bulla, M., and Fürst, P.: The effect of low-dose supplementation of L-carnitine on lipid metabolism in hemodialyzed children. Kidney Int. in press (1989).Google Scholar
  39. 40.
    D’Attellis, N.P., Kulapongse, S., Richelle, M., Dahlan, W., Elwyn, D.H., Rössle, C., Fürst, P., and Carpentier, Y.: Metabolic utilization of energy substrates during intravenous L-carnitine supplementation in normal man. Metabolism in press (1989).Google Scholar
  40. 41.
    Sandstedt, S., Cederblad, G. , Cennmarken, C., Lindholm, M., Larsson, J.: The effect of L-carnitine supplemented TPN on lipid- and nitrogen-metabolism in critically ill patients. Clin. Nutr. 6 (spec, suppl.):24 (1987).CrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1989

Authors and Affiliations

  • Peter Fürst
    • 1
  • Anne Glöggler
    • 1
  • Claudia Rössle
    • 1
    • 2
  1. 1.Institut für Biologische Chemie und ErnährungswissenschaftUniversität HohenheimStuttgart 70Deutschland
  2. 2.Laboratoire de NutritionHopital St. PierreBruxellesBelgium

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