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Serum Carnitine: An Index of Muscle Destruction in Man

Serum Carnitine: An Index of Muscle Destruction in Man Abstract Carnitine was identified as a constituent of normal human serum by reaction with a specific enzyme and by thin-layer chromatography. A spectrophotometric enzymatic assay was adapted for use with human serum, based upon the liberation of coenzyme A from acetylcoenzyme A in the presence of carnitine and carnitine acetyltransferase. Normal human serum contains 2.3μmols to 7.0μmols/100ml. Serum content increased to 33.4μmols/100 ml during attacks of myoglobinuria but was not increased in patients with Duchenne dystrophy, liver disease, uremia, or acute myocardial infarction. References 1. Pearson DJ, Chase JFA, Tubbs PK: The assay of (—)-carnitine and its O-acyl derivatives . Methods Enzymol 14:612-622, 1969. 2. Lindstedt G, Lindstedt S: The separation of carnitine from related compounds by ion-exchange and thin layer chromatography , Wolf G (ed): in Recent Research on Carnitine . Cambridge Mass, MIT Press, 1965, p 11-21. 3. Marquis NR, Fritz IB: The distribution of carnitine, acetylcarnitine, and carnitine acetyl-transferase in rat tissues . J Biol Chem 240:2193-2196, 1965. 4. Lindstedt G, Lindstedt S: On the biosynthesis and degradation of carnitine . Biochem Biophys Res Comm 6:319-323, 1962.Crossref 5. Lindstedt G: Hydroxylation of γ-butyrobetaine to carnitine in rat liver . Biochemistry 6:1271-1282, 1967.Crossref 6. Bremer J: Carnitine precursors in the rat . Biochim Biophys Acta 57:327-335, 1962.Crossref 7. Wolf G, Berger CRA: Studies on the biosynthesis and turnover of carnitine . Arch Biochem Biophys 92:360-365, 1961.Crossref 8. Cederblad G, Lindstedt S: Excretion of Lcarnitine in man . Clin Chim Acta 33:117-123, 1971.Crossref 9. Lindstedt S, Lindstedt G: Distribution and excretion of carnitine in the rat . Acta Chem Scand 15:701-702, 1961.Crossref 10. Yue KTN, Fritz IB: Fate of tritium-labeled carnitine administered to dogs and rats . Am J Physiol 202:122-128, 1962. 11. Khairallah EA, Wolf G: Carnitine decarboxylase: The conversion of carnitine to β-methylcholine . J Biol Chem 242:32-39, 1967. 12. Mehlman MA, Abdel Kader MM, Therriault DG: Metabolism, turnover time, half-life, body pool of carnitine-14C in normal, alloxan diabetic and insulin-treated rats . Life Sci 8:465-472, 1969.Crossref 13. Strack E, Benn H, Rotzsch W: Trimethylamin und Trimethylaminoxyd im Harn nach Zufuhr von 15N und 15N (—)-Carnitin . Acta Biol Med Germ 11:14-28, 1963. 14. Bieber LL, Sellers LG, Kumar SS: Studies on the formation and degradation of phosphatidyl-β-methylcholine, β-methylcholine derivatives, and carnitine by housefly larvae . J Biol Chem 244:630-636, 1969. 15. Blum K, Seifter E, Seifter J: The pharmacology of d- and l-carnitine and d- and l-acetylcarnitine: Comparison with choline and acetylcholine . J Pharmacol Exp Ther 178:331-338, 1971. 16. Soderberg J, Therriault DG, Wolf G: Characterization of lipid-bound carnitine , in Wolf G (ed): Recent Research on Carnitine . Cambridge, Mass, MIT Press, 1965, pp 165-172. 17. Bremer J: Carnitine in intermediary metabolism: Reversible acetylation of carnitine by mitochondria . J Biol Chem 237:2228-2231, 1962. 18. Klingenberg M, Bode C: Some aspects of the role of carnitine in fatty acid oxidation , in Wolf G (ed): Recent Research on Carnitine . Cambridge, Mass, 1965, pp 87-95. 19. Fritz IB: An hypothesis concerning the role of carnitine in the control of interrelations between fatty acid and carbohydrate metabolism . Perspect Biol Med 10:643-677, 1967. 20. Bressler R: Carnitine and the twins . N Engl J Med 282:745-746, 1970.Crossref 21. Beenakkers AMT, Henderson PT: The localization and function of carnitine acetyltransferase in the flight muscles of the locust . Europ J Biochem 1:187-192, 1967.Crossref 22. Yates DW, Garland PB: Carnitine palmitoyltransferase activities of rat liver mitochondria . Biochem J 119:547-552, 1970. 23. Bohmer T, Norum KR, Bremer J: The relative amounts of long-chain acylcarnitine, acetyl carnitine, and free carnitine in organs of rats in different nutritional states and with alloxan diabetes . Biochim Biophys Acta 125:244-251, 1966.Crossref 24. Bode C, et al: Effects of ethanol on free coenzyme A, free carnitine and their fatty acid esters in rat liver . Biochim Biophys Acta 210:448-455, 1970.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Serum Carnitine: An Index of Muscle Destruction in Man

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References (23)

Publisher
American Medical Association
Copyright
Copyright © 1973 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1973.00490210066009
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Abstract

Abstract Carnitine was identified as a constituent of normal human serum by reaction with a specific enzyme and by thin-layer chromatography. A spectrophotometric enzymatic assay was adapted for use with human serum, based upon the liberation of coenzyme A from acetylcoenzyme A in the presence of carnitine and carnitine acetyltransferase. Normal human serum contains 2.3μmols to 7.0μmols/100ml. Serum content increased to 33.4μmols/100 ml during attacks of myoglobinuria but was not increased in patients with Duchenne dystrophy, liver disease, uremia, or acute myocardial infarction. References 1. Pearson DJ, Chase JFA, Tubbs PK: The assay of (—)-carnitine and its O-acyl derivatives . Methods Enzymol 14:612-622, 1969. 2. Lindstedt G, Lindstedt S: The separation of carnitine from related compounds by ion-exchange and thin layer chromatography , Wolf G (ed): in Recent Research on Carnitine . Cambridge Mass, MIT Press, 1965, p 11-21. 3. Marquis NR, Fritz IB: The distribution of carnitine, acetylcarnitine, and carnitine acetyl-transferase in rat tissues . J Biol Chem 240:2193-2196, 1965. 4. Lindstedt G, Lindstedt S: On the biosynthesis and degradation of carnitine . Biochem Biophys Res Comm 6:319-323, 1962.Crossref 5. Lindstedt G: Hydroxylation of γ-butyrobetaine to carnitine in rat liver . Biochemistry 6:1271-1282, 1967.Crossref 6. Bremer J: Carnitine precursors in the rat . Biochim Biophys Acta 57:327-335, 1962.Crossref 7. Wolf G, Berger CRA: Studies on the biosynthesis and turnover of carnitine . Arch Biochem Biophys 92:360-365, 1961.Crossref 8. Cederblad G, Lindstedt S: Excretion of Lcarnitine in man . Clin Chim Acta 33:117-123, 1971.Crossref 9. Lindstedt S, Lindstedt G: Distribution and excretion of carnitine in the rat . Acta Chem Scand 15:701-702, 1961.Crossref 10. Yue KTN, Fritz IB: Fate of tritium-labeled carnitine administered to dogs and rats . Am J Physiol 202:122-128, 1962. 11. Khairallah EA, Wolf G: Carnitine decarboxylase: The conversion of carnitine to β-methylcholine . J Biol Chem 242:32-39, 1967. 12. Mehlman MA, Abdel Kader MM, Therriault DG: Metabolism, turnover time, half-life, body pool of carnitine-14C in normal, alloxan diabetic and insulin-treated rats . Life Sci 8:465-472, 1969.Crossref 13. Strack E, Benn H, Rotzsch W: Trimethylamin und Trimethylaminoxyd im Harn nach Zufuhr von 15N und 15N (—)-Carnitin . Acta Biol Med Germ 11:14-28, 1963. 14. Bieber LL, Sellers LG, Kumar SS: Studies on the formation and degradation of phosphatidyl-β-methylcholine, β-methylcholine derivatives, and carnitine by housefly larvae . J Biol Chem 244:630-636, 1969. 15. Blum K, Seifter E, Seifter J: The pharmacology of d- and l-carnitine and d- and l-acetylcarnitine: Comparison with choline and acetylcholine . J Pharmacol Exp Ther 178:331-338, 1971. 16. Soderberg J, Therriault DG, Wolf G: Characterization of lipid-bound carnitine , in Wolf G (ed): Recent Research on Carnitine . Cambridge, Mass, MIT Press, 1965, pp 165-172. 17. Bremer J: Carnitine in intermediary metabolism: Reversible acetylation of carnitine by mitochondria . J Biol Chem 237:2228-2231, 1962. 18. Klingenberg M, Bode C: Some aspects of the role of carnitine in fatty acid oxidation , in Wolf G (ed): Recent Research on Carnitine . Cambridge, Mass, 1965, pp 87-95. 19. Fritz IB: An hypothesis concerning the role of carnitine in the control of interrelations between fatty acid and carbohydrate metabolism . Perspect Biol Med 10:643-677, 1967. 20. Bressler R: Carnitine and the twins . N Engl J Med 282:745-746, 1970.Crossref 21. Beenakkers AMT, Henderson PT: The localization and function of carnitine acetyltransferase in the flight muscles of the locust . Europ J Biochem 1:187-192, 1967.Crossref 22. Yates DW, Garland PB: Carnitine palmitoyltransferase activities of rat liver mitochondria . Biochem J 119:547-552, 1970. 23. Bohmer T, Norum KR, Bremer J: The relative amounts of long-chain acylcarnitine, acetyl carnitine, and free carnitine in organs of rats in different nutritional states and with alloxan diabetes . Biochim Biophys Acta 125:244-251, 1966.Crossref 24. Bode C, et al: Effects of ethanol on free coenzyme A, free carnitine and their fatty acid esters in rat liver . Biochim Biophys Acta 210:448-455, 1970.Crossref

Journal

Archives of Neurology – American Medical Association

Published: Mar 1, 1973

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