Myocardial function and energy metabolism in carnitine-deficient rats

Cellular and Molecular Life Sciences

Volumn 60, Issue 4, 2003, Pages 767-775

  Zaugg, C E ^a   Spaniol, M ^b   Kaufmann, P ^b   Bellahcene, M ^a   Barbosa, V ^a,c   Tolnay, M ^d   Buser, P T ^c   Krahenbuhl S ^b  

^a UNIVERSITY HOSPITAL BASEL   (Switzerland)

^b Div of Clin Pharmacol/Toxicology   (Switzerland)

^c Division of Cardiology   (Switzerland)

^d UNIVERSITY HOSPITAL BASEL   (Switzerland)

Author keywords

Cardiomyopathy; Contractile function; Fatty acid; Glycogen; Mitochondria; N trimethyl hydrazine 3 propionate.; Oxidative phosphorylation; Systemic carnitine deficiency

Indexed keywords

CARNITINE; CARNITINE PALMITOYLTRANSFERASE; CITRATE SYNTHASE; CYTOCHROME C OXIDASE; GLUCOSE; GLYCOGEN; LONG CHAIN FATTY ACID; MILDRONATE; N TRIMETHYLHYDRAZINE 3 PROPIONATE; PALMITIC ACID; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CARDIOMYOPATHY; CARNITINE DEFICIENCY; CONTROLLED STUDY; DISEASE ASSOCIATION; ENERGY METABOLISM; ENERGY YIELD; ENZYME ACTIVITY; FATTY ACID SYNTHESIS; FORCE; FREQUENCY DISCRIMINATION; GLYCOGEN METABOLISM; HEART FUNCTION; HEART LEFT VENTRICLE CONTRACTION; HEART MUSCLE CONTRACTILITY; HEART VOLUME; HISTOPATHOLOGY; ISOLATED HEART; MITOCHONDRIAL RESPIRATION; NONHUMAN; RAT; SYSTOLIC BLOOD PRESSURE;

ADENOSINE TRIPHOSPHATE; ANIMALS; CARNITINE; GLYCOGEN; HEART; MITOCHONDRIA; MYOCARDIUM; PHOSPHOCREATINE; RATS;

ANIMALIA;

EID: 0038026038     PISSN: 1420682X     EISSN: None     Source Type: Journal    
DOI: 10.1007/s00018-003-3011-1     Document Type: Article

References (34)

1. Bremer J. (1983) Carnitine - metabolism and functions. Physiol. Rev. 63: 1420-1480
2. Broderick T. L., Quinney H. A. and Lopaschuk G. D. (1992) Carnitine stimulation of glucose oxidation in the fatty acid perfused isolated working rat heart. J. Biol. Chem. 267: 3758-3763
3. Bremer J. (1969) Pyruvate dehydrogenase, substrate specificity and product inhibition. Eur. J. Biochem. 8: 535-540
4. Lango R., Smolenski R. T., Narkiewicz M., Suchorzewska J. and Lysiak-Szydlowska W. (2001) Influence of L-carnitine and its derivatives on myocardial metabolism and function in ischemic heart disease and during cardiopulmonary bypass. Cardiovasc. Res. 51: 21-29
5. Brass E. P. and Hoppel C. L (1980) Relationship between acid-soluble carnitine and coenzyme A pools in vivo. Biochem. J. 190: 495-504
6. Gilbert E. F (1985) Carnitine deficiency. Pathology 17: 161-171
7. Wynne J. and Braunwald E (1997) The cardiomyopathies and myocarditides. In: Heart Disease: A Textbook of Cardiovascular Medicine, 5th edn, pp. 1404-1463, Braunwald E. (ed.), Saunders, Philadelphia
8. Winter S., Jue K., Prochazka J., Francis P., Hamilton W., Linn L. et al. (1995) The role of L-carnitine in pediatric cardiomyopathy. J. Child Neurol. 10 (Suppl 2): S45-S51
9. Paulson D. J. (1998) Carnitine deficiency-induced cardiomyopathy. Mol. Cell. Biochem 180: 33-41
10. Spaniol M., Brooks H., Auer L., Zimmermann A., Solioz M., Stieger B. et al. (2001) Development and characterization of an animal model of carnitine deficiency. Eur. J. Biochem. 268: 1876-1887
11. Simkhovich B. Z., Shutenko Z. V., Meirena D. V., Khagi K. B., Mezapuke R. J., Molodchina T. N. et al. (1988) 3-(2,2,2-Trimethylhydrazinium)propionate (THP) - a novel gammabutyrobetaine hydroxylase inhibitor with cardioprotective properties. Biochem. Pharmacol. 37: 195-202
12. Zaugg C. E., Kojima S., Wu S. T., Wikman-Coffelt J., Parmley W. W. and Buser P. T. (1995) Intracellular calcium transients underlying interval-force relationship in whole rat hearts: effects of calcium antagonists. Cardiovasc. Res. 30: 212-221
13. Soares P. R., Albuquerque C. P. de, Chacko V. P., Gerstenblith G. and Weiss R. G. (1997) Role of preischemic glycogen depletion in the improvement of postischemic metabolic and contractile recovery of ischemia-preconditioned rat hearts. Circulation 96: 975-983
14. Barbosa V., Sievers R. E., Zaugg C. E. and Wolfe C. L. (1996) Preconditioning ischemia time determines the degree of glycogen depletion and infarct size reduction in rat hearts. Am. Heart J. 131: 224-230
15. Brass E. P. and Hoppel C. L. (1978) Carnitine metabolism in the fasting rat. J. Biol. Chem. 253: 2688-2693
16. Gilbert J. C. and Glantz S. A. (1989) Determinants of left ventricular filling and of the diastolic pressure-volume relation. Circ. Res. 64: 827-852
17. Tamiya K., Beppu T. and Ishihara K. (1995) Double-exponential curve fitting of isometric relaxation: a new measure for myocardial lusitropism. Am. J. Physiol. 269: H393-H406
18. 2+] staircase in the isovolumic pressure-frequency relationship of Langendorff-perfused rat heart. J. Mol. Cell. Cardiol. 28: 65-77
19. Srere P. A. (1969) Citrate synthase. Methods Enzymol. 13: 3-11
20. Krähenbühl S., Chang M., Brass E. P. and Hoppel C. L. (1991) Decreased activities of ubiquinol:ferricytochrome c oxidoreductase (complex III) and ferrocytochrome c:oxygen oxidoreductase (complex IV) in liver mitochondria from rats with hydroxycobalamin[c-lactam]-induced methylmalonic aciduria. J. Biol. Chem. 266: 20998-21003
21. Bremer J. (1981) The effect of fasting on the activity of liver carnitine palmitoyltransferase and its inhibition by malonyl-CoA. Biochim. Biophys. Acta 665: 628-631
22. Adams H. (1963) Adenosin-5′-triphosphate, determination with phosphoglycerate kinase. In: Methods of Enzymatic analysis, pp. 539-543, Bergmayer H. U. (ed.) Academic Press, New York
23. Willer B., Stucki G., Hoppeler H., Bruhlmann P. and Krahenbuhl S. (2000) Effects of creatine supplementation on muscle weakness in patients with rheumatoid arthritis. Rheumatology 39: 293-298
24. Olsen C. (1971) An enzymatic fluorimetric micromethod for the determination of acetoacetate, beta-hydroxybutyrate, pyruvate and lactate. Clin. Chim. Acta 33: 293-300
25. Dawson-Saunders B. and Trapp R. G. (1990) Basic and Clinical Biostatistics, Appleton & Lange, San Mateo, Calif.
26. Paulson D. J. (1998) Secondary L-carnitine deficiency-induced cardiomyopathy. Cardiologia 43 (Suppl 2): 677-683
27. Wood E. H., Hepner R. L. and Weidmann S. (1969) Inotropic effects of electrical currents. Circ. Res. 24: 409-445
28. Spaniol M., Kaufmann P., Beier K., Wuthrich J., Torok M., Scharnagl H. et al. (2003) Mechanisms of liver steatosis in rats with systemic carnitine deficiency due to treatment with trimethylhydraziniumpropionate. J. Lipid Res. 44: 144-153
29. Faergeman N. J. and Knudsen J. (1997) Role of long-chain fatty acyl-CoA esters in the regulation of metabolism and in cell signalling. Biochem. J. 323: 1-12
30. Brown N. F., Weis B. C., Husti J. E., Foster D. W. and McGarry J. D. (1995) Mitochondrial carnitine palmitoyltransferase I isoform switching in the developing rat heart. J. Biol. Chem. 270: 8952-8957
31. McGarry J. D., Mills S. E., Long C. S. and Foster D. W. (1983) Observations on the affinity for carnitine, and malonyl-CoA sensitivity, of carnitine palmitoyltransferase I in animal and human tissues: demonstration of the presence of malonyl-CoA in non-hepatic tissues of the rat. Biochem. J. 214: 21-28
32. Haller R. G., Lewis S. F., Estabrook R. W., DiMauro S., Servidei S. and Foster D. W. (1989) Exercise intolerance, lactic acidosis, and abnormal cardiopulmonary regulation in exercise associated with adult skeletal muscle cytochrome c oxidase deficiency. J. Clin. Invest. 84: 155-161
33. Hoppel C. L., Kerr D. S., Dahms B. and Roessmann U. (1987) Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport: fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy. J. Clin. Invest. 80: 71-77
34. Horiuchi M., Yoshida H., Kobayashi K., Kuriwaki K., Yoshimine K., Tomomura M. et al. (1993) Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. FEBS Lett. 326: 267-271