Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice

Journal of Lipid Research

Volumn 50, Issue 8, 2009, Pages 1556-1562

  ter Veld, Frank ^a   Primassin, Sonja ^a   Hoffmann, Lars ^a   Mayatepek, Ertan ^a   Spiekerkoetter, Ute ^a  

^a HEINRICH HEINE UNIVERSITY   (Germany)

Author keywords

oxidation; Acyl coenzyme A; Acylcarnitine; Very long chain acyl CoA dehydrogenase

Indexed keywords

ACYLCARNITINE; CARNITINE; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; VERY LONG CHAIN FATTY ACID; ACYL COENZYME A; DRUG DERIVATIVE;

ACCURACY; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; ESSENTIAL FATTY ACID DEFICIENCY; FATTY ACID ANALYSIS; FATTY ACID OXIDATION; KNOCKOUT MOUSE; MOUSE; MUSCLE STRESS; MUSCLE TISSUE; NONHUMAN; PRIORITY JOURNAL; TANDEM MASS SPECTROMETRY; TREADMILL EXERCISE; WILD TYPE; ANIMAL; BODY WEIGHT; C57BL MOUSE; CHEMISTRY; ENZYMOLOGY; EXERCISE; GENETICS; MOUSE MUTANT; MUSCLE MITOCHONDRION; PHYSIOLOGY; SKELETAL MUSCLE;

ACYL COENZYME A; ACYL-COA DEHYDROGENASE, LONG-CHAIN; ANIMALS; BODY WEIGHT; CARNITINE; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MITOCHONDRIA, MUSCLE; MUSCLE, SKELETAL; PHYSICAL EXERTION;

EID: 69449089762     PISSN: 00222275     EISSN: 15397262     Source Type: Journal    
DOI: 10.1194/jlr.M800221-JLR200     Document Type: Article

References (30)

1. Wanders, R. J., P. Vreken, M. E. den Boer, F. A. Wijburg, A. H. van Gennip, and L. Ijlst. 1999. Disorders of mitochondrial fatty acyl-CoA beta-oxidation. J. Inherit. Metab. Dis. 22: 442-487.
2. Mathur, A., H. F. Sims, D. Gopalakrishnan, B. Gibson, P. Rinaldo, J. Vockley, G. Hug, and A. W. Strauss. 1999. Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death. Circulation. 99: 1337-1343.
3. Strauss, A. W., C. K. Powell, D. E. Hale, M. M. Anderson, A. Ahuja, J. C. Brackett, and H. F. Sims. 1995. Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood. Proc. Natl. Acad. Sci. USA. 92: 10496-10500.
4. Liebig, M., I. Schymik, M. Mueller, U. Wendel, E. Mayatepek, J. Ruiter, A. W. Strauss, R. J. Wanders, and U. Spiekerkoetter. 2006. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels. Pediatrics. 118: 1065-1069.
5. Schymik, I., M. Liebig, M. Mueller, U. Wendel, E. Mayatepek, A. W. Strauss, R. J. Wanders, and U. Spiekerkoetter. 2006. Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry. J. Pediatr. 149: 128-130.
6. Vianey-Saban, C., P. Divry, M. Brivet, M. Nada, M. T. Zabot, M. Mathieu, and C. Roe. 1998. Mitochondrial very-long-chain acylcoenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients. Clin. Chim. Acta. 269: 43-62.
7. Exil, V. J., R. L. Roberts, H. Sims, J. E. McLaughlin, R. A. Malkin, C. D. Gardner, G. Ni, J. N. Rottman, and A. W. Strauss. 2003. Very-long-chain acyl-coenzyme a dehydrogenase deficiency in mice. Circ. Res. 93: 448-455.
8. Spiekerkoetter, U., C. Tokunaga, U. Wendel, E. Mayatepek, V. Exil, M. Duran, F. A. Wijburg, R. J. Wanders, and A. W. Strauss. 2004. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress. Eur. J. Clin. Invest. 34: 191-196.
9. Werdich, A. A., F. Baudenbacher, I. Dzhura, L. H. Jeyakumar, P. J. Kannankeril, S. Fleischer, A. LeGrone, D. Milatovic, M. Aschner, A. W. Strauss, et al. 2007. Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice. Am. J. Physiol. Heart Circ. Physiol. 292: H2202-H2211.
10. Exil, V. J., C. D. Gardner, J. N. Rottman, H. Sims, B. Bartelds, Z. Khuchua, R. Sindhal, G. Ni, and A. W. Strauss. 2006. Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase. Am. J. Physiol. Heart Circ. Physiol. 290: H1289-H1297.
11. Liebig, M., M. Gyenes, G. Brauers, J. P. Ruiter, U. Wendel, E. Mayatepek, A. W. Strauss, R. J. Wanders, and U. Spiekerkoetter. 2006. Carnitine supplementation induces long-chain acylcarnitine production-studies in the VLCAD-deficient mouse. J. Inherit. Metab. Dis. 29: 343-344.
12. Primassin, S., F. ter Veld, E. Mayatepek, and U. Spiekerkoetter. 2008. Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine. Pediatr. Res. 63: 632-637.
13. Bonnet, D., D. Martin, D. L. Pascale, E. Villain, P. Jouvet, D. Rabier, M. Brivet, and J. M. Saudubray. 1999. Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. Circulation. 100: 2248-2253.
14. Spiekerkoetter, U., B. Sun, T. Zytkovicz, R. Wanders, A. W. Strauss, and U. Wendel. 2003. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase defi ciency. J. Pediatr. 143: 335-342.
15. Ciapaite, J., S. J. Bakker, M. Diamant, G. van Eikenhorst, R. J. Heine, H. V. Westerhoff, and K. Krab. 2006. Metabolic control of mitochondrial properties by adenine nucleotide translocator determines palmitoyl-CoA effects. Implications for a mechanism linking obesity and type 2 diabetes. FEBS J. 273: 5288-5302.
16. Spiekerkoetter, U., C. Tokunaga, U. Wendel, E. Mayatepek, L. Ijlst, F. M. Vaz, N. van Vlies, H. Overmars, M. Duran, F. A. Wijburg, et al. 2005. Tissue carnitine homeostasis in very-longchain acyl-CoA dehydrogenase-deficient mice. Pediatr. Res. 57: 760-764.
17. van Vlies, N., L. Tian, H. Overmars, A. H. Bootsma, W. Kulik, R. J. Wanders, P. A. Wood, and F. M. Vaz. 2005. Characterization of carnitine and fatty acid metabolism in the long-chain acyl-CoA dehydrogenase-deficient mouse. Biochem. J. 387: 185-193.
18. Shepherd, D., and P. B. Garland. 1969. The kinetic properties of citrate synthase from rat liver mitochondria. Biochem. J. 114: 597-610.
19. Schafer, C., L. Hoffmann, K. Heldt, M. R. Lornejad-Schafer, G. Brauers, T. Gehrmann, T. A. Garrow, D. Haussinger, E. Mayatepek, B. C. Schwahn, et al. 2007. Osmotic regulation of betaine homocysteine-S-methyltransferase expression in H4IIE rat hepatoma cells. Am. J. Physiol. Gastrointest. Liver Physiol. 292: G1089-G1098.
20. Smelt, A. H., B. J. Poorthuis, W. Onkenhout, H. R. Scholte, B. S. Andresen, S. G. van Duinen, N. Gregersen, and A. R. Wintzen. 1998. Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset. Ann. Neurol. 43: 540-544.
21. Idell-Wenger, J. A., L. W. Grotyohann, and J. R. Neely. 1978. Coenzyme A and carnitine distribution in normal and ischemic hearts. J. Biol. Chem. 253: 4310-4318.
22. Jeneson, J. A., M. W. de Snoo, N. A. Verlinden, B. J. Joosten, A. Doornenbal, A. Schot, and M. E. Everts. 2007. Treadmill but not wheel running improves fatigue resistance of isolated extensor digitorum longus muscle in mice. Acta Physiol. (Oxf.). 190: 151-161.
23. Cox, K. B., D. A. Hamm, D. S. Millington, D. Matern, J. Vockley, P. Rinaldo, C. A. Pinkert, W. J. Rhead, J. R. Lindsey, and P. A. Wood. 2001. Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse. Hum. Mol. Genet. 10: 2069-2077.
24. Ciapaite, J., S. J. L. Bakker, G. van Eikenhorst, M. J. Wagner, T. Teerlink, C. G. Schalkwijk, M. Fodor, D. M. Ouwens, M. Diamante, R. J. Heine, et al. 2007. Functioning of oxidative phosphorylation in liver mitochondria of high-fat diet fed rats. Biochim. Biophys. Acta. 1772: 307-316.
25. Kalderon, B., V. Sheena, S. Shachrur, R. Hertz, and J. Bar-Tana. 2002. Modulation by nutrients and drugs of liver acyl-CoAs analyzed by mass spectrometry. J. Lipid Res. 43: 1125-1132.
26. Sun, D., M. G. Cree, and R. R. Wolfe. 2006. Quantification of the concentration and 13C tracer enrichment of long-chain fatty acylcoenzyme A in muscle by liquid chromatography/mass spectrometry. Anal. Biochem. 349: 87-95.
27. Woldegiorgis, G., T. Spennetta, B. E. Corkey, J. R. Williamson, and E. Shrago. 1985. Extraction of tissue long-chain acyl-CoA esters and measurement by reverse-phase high-performance liquid chromatography. Anal. Biochem. 150: 8-12.
28. Yu, C., Y. Chen, G. W. Cline, D. Zhang, H. Zong, Y. Wang, R. Bergeron, J. K. Kim, S. W. Cushman, G. J. Cooney, et al. 2002. Mechanism by which fatty acids inhibit insulin activation of insulin receptor substrate-1 (IRS-1)-associated phosphatidylinositol 3-kinase activity in muscle. J. Biol. Chem. 277: 50230-50236.
29. Ramsay, R. R., and A. Arduini. 1993. The carnitine acyltransferases and their role in modulating acyl-CoA pools. Arch. Biochem. Biophys. 302: 307-314.
30. Faergeman, N. J., and J. Knudsen. 1997. Role of long-chain fatty acyl-CoA esters in the regulation of metabolism and in cell signalling. Biochem. J. 323: 1-12.