Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects

Molecular Genetics and Metabolism

Volumn 86, Issue 3, 2005, Pages 344-352

  Ventura, F V ^a,b   Ruiter, J ^b   Ijlst, L ^b   Tavares De Almeida, I ^a   Wanders, R J A ^b  

^a RESEARCH INSTITUTE FOR MEDICINES IMED ULISBOA   (Portugal)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

Author keywords

ATP ADP carrier; Dicarboxylate carrier; Glutamate carrier; Hypoglycemia; Lactic acidemia; Long chain acyl CoA esters; Mitochondrial fatty acid oxidation; Mitochondrial long chain fatty acid oxidation deficiencies; Oxidative phosphorylation

Indexed keywords

2,3 UNSATURATED PALMITOYL COENZYME A; 2,4 DINITROPHENOL; 3 HYDROXYPALMITOYL COENZYME A; 3 KETOPALMITOYL COENZYME A; ACYL COENZYME A; ADENOSINE DIPHOSPHATE; ADENOSINE TRIPHOSPHATE; DICARBOXYLATE TRANSPORTER; GLUTAMIC ACID; HYDROXYL GROUP; LONG CHAIN FATTY ACID; MALONIC ACID DERIVATIVE; N BUTYLMALONATE; PALMITOYL COENZYME A; PHOSPHATE; ROTENONE; SUCCINIC ACID; UNCLASSIFIED DRUG;

AMINO ACID TRANSPORT; ANIMAL CELL; ARTICLE; CONTROLLED STUDY; FATTY ACID OXIDATION; LIVER MITOCHONDRION; MALE; METABOLIC DISORDER; MITOCHONDRIAL MEMBRANE; MITOCHONDRIAL RESPIRATION; MITOCHONDRION SWELLING; NONHUMAN; OXYGEN CONSUMPTION; PRIORITY JOURNAL; PURINE SYNTHESIS; RAT; TRANSPORT KINETICS;

ACYL COENZYME A; ADENOSINE TRIPHOSPHATE; ANIMALS; BIOLOGICAL TRANSPORT; DICARBOXYLIC ACID TRANSPORTERS; DRUG SYNERGISM; ESTERS; FATTY ACIDS; GLUTAMIC ACID; MALE; MALONATES; MITOCHONDRIA, LIVER; MITOCHONDRIAL SWELLING; OXIDATION-REDUCTION; OXIDATIVE PHOSPHORYLATION; OXYGEN CONSUMPTION; RATS; RATS, WISTAR; SUCCINIC ACID;

ANIMALIA;

EID: 27644437646     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2005.07.030     Document Type: Article

References (42)

1. S. Eaton Control of mitochondrial beta-oxidation flux Prog. Lipid Res. 41 2002 197 239
2. R.J. Wanders, P. Vreken, M.E. den Boer, F.A. Wijburg, A.H. van Gennip, and L. IJlst Disorders of mitochondrial fatty acyl-CoA beta-oxidation J. Inherit. Metab. Dis. 22 1999 442 487
3. C. Roe, and J. Ding Mitochondrial fatty acid oxidation disorders C.R. Scriver A.L. Beaudet W.S. Sly D. Valle The Metabolic and Molecular Bases of Inherited Disease eighth ed. 2001 McGraw-Hill New York 2297 2326
4. R. Parini, B. Garavaglia, J.M. Saudubray, P. Bardelli, D. Melotti, G. Zecca, and S. Di Donato Clinical diagnosis of long-chain acyl-coenzyme A-dehydrogenase deficiency: use of stress and fat-loading tests J. Pediatr. 119 1991 77 80
5. J.M. Saudubray, G. Mitchell, J.P. Bonnefont, G. Schwartz, C. Nuttin, A. Munnich, M. Brivet, A. Vassault, F. Demaugre, and D. Rabier Approach to the patient with a fatty acid oxidation disorder Prog. Clin. Biol. Res. 375 1992 271 288
6. S. Yamaguchi, Y. Indo, P.M. Coates, T. Hashimoto, and K. Tanaka Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency Pediatr. Res. 34 1993 111 113
7. C. Bertrand, C. Largilliere, M.T. Zabot, M. Mathieu, and C. Vianey-Saban Very long chain acyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid oxidation in fibroblasts Biochim. Biophys. Acta 1180 1993 327 329
8. R.J. Pollitt Disorders of mitochondrial beta-oxidation: prenatal and early postnatal diagnosis and their relevance to Reye's syndrome and sudden infant death J. Inherit. Metab. Dis. 12 1989 215 230
9. S. Jackson, R.S. Kler, K. Bartlett, H. Briggs, L.A. Bindoff, M. Pourfarzam, D. Gardner-Medwin, and D.M. Turnbull Combined enzyme defect of mitochondrial fatty acid oxidation J. Clin. Invest. 90 1992 1219 1225
10. M. Pourfarzam, J. Schaefer, D.M. Turnbull, and K. Bartlett Analysis of fatty acid oxidation intermediates in cultured fibroblasts to detect mitochondrial oxidation disorders Clin. Chem. 40 1994 2267 2275
11. R.J. Pollitt Disorders of mitochondrial long-chain fatty acid oxidation J. Inherit. Metab. Dis. 18 1995 473 490
12. P.V. Vignais Molecular and physiological aspects of adenine nucleotide transport in mitochondria Biochim. Biophys. Acta 456 1976 1 38
13. L. Wojtczak Effect of long-chain fatty acids and acyl-CoA on mitochondrial permeability, transport, and energy-coupling processes J. Bioenerg. Biomembr. 8 1976 293 311
14. J. Ciapaite, G. van Eikenhorst, and K. Krab Application of modular control analysis to inhibition of the adenine nucleotide translocator by palmitoyl-CoA Mol. Biol. Rep. 29 2002 13 16
15. E. Bertini, C. Dionisi-Vici, B. Garavaglia, A.B. Burlina, M. Sabatelli, M. Rimoldi, A. Bartuli, G. Sabetta, and S. DiDonato Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxyacylCoA dehydrogenase deficiency Eur. J. Pediatr. 51 1992 121 126
16. D.E. Hale, and M.J. Bennett Fatty acid oxidation disorders: a new class of metabolic diseases J. Pediatr. 121 1992 1 11
17. M. Duran, L. Bruinvis, D. Ketting, and L. Dorland Diagnosis of mitochondrial fatty acid oxidation defects Padiatr. Padol. 28 1993 19 25
18. R. Moore, J.F. Glasgow, M.A. Bingham, J.A. Dodge, R.J. Pollitt, S.E. Olpin, B. Middleton, and K. Carpenter Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency-diagnosis, plasma carnitine fractions and management in a further patient Eur. J. Pediatr. 152 1993 433 436
19. W.R. Treem, P. Rinaldo, D.E. Hale, C.A. Stanley, D.S. Millington, J.S. Hyams, S. Jackson, and D.M. Turnbull Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency Hepatology 19 1994 339 345
20. T. Tyni, A. Majander, H. Kalimo, J. Rapola, and H. Pihko Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation Neuromuscul. Disord. 6 1996 327 337
21. T. Tyni, A. Palotie, L. Viinikka, L. Valanne, M.K. Salo, U. von Dobeln, S. Jackson, R. Wanders, N. Venizelos, and H. Pihko Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency with the G1528C mutation: clinical presentation of thirteen patients J. Pediatr. 130 1997 67 76
22. J.A. Ibdah, M.J. Dasouki, and A.W. Strauss Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia J. Inherit. Metab. Dis. 22 1999 811 814
23. J.A. Ibdah, M.J. Bennett, P. Rinaldo, Y. Zhao, B. Gibson, H.F. Sims, and A.W. Strauss A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women N. Engl. J. Med. 340 1999 1723 1731
24. A. Chakrapani, S. Olpin, M. Cleary, J.H. Walter, J.E. Wraith, and G.T. Besley Trifunctional protein deficiency: three families with significant maternal hepatic dysfunction in pregnancy not associated with E474Q mutation J. Inherit. Metab. Dis. 23 2000 826 834
25. U. Spiekerkoetter, B. Sun, Z. Khuchua, M.J. Bennett, and A.W. Strauss Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations Hum. Mutat. 21 2003 598 607
26. M.E. den Boer, C. Dionisi-Vici, A. Chakrapani, A.O. van Thuijl, R.J. Wanders, and F.A. Wijburg Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement J. Pediatr. 142 2003 684 689
27. J.E. Lee, H.R. Yoon, K.H. Paik, S.J. Hwang, J.W. Shim, Y.S. Chang, W.S. Park, A.W. Strauss, and D.K. Jin A case of mitochondrial trifunctional protein deficiency diagnosed by acylcarnitine profile and DNA analysis in a dried blood spot of a 4-day-old boy J. Inherit. Metab. Dis. 26 2003 403 406
28. J.E. Ireland, B.A. Heese, A.W. Strauss, V.V. Michels, D. Whiteman, S.H. Hahn, P. Rinaldo, and D. Matern Severe lactic acidosis in the absence of dicarboxylic aciduria in a newborn with mitochondrial trifunctional protein deficiency (MTP) J. Inherit. Metab. Dis. 27 2004 107
29. F.V. Ventura, J.P.N. Ruiter, L. IJlst, I.T. Almeida, and R.J. Wanders Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders Biochim. Biophys. Acta 1272 1995 14 20
30. F.V. Ventura, J.P.N. Ruiter, L. IJlst, I.T. de Almeida, and R.J. Wanders Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acid beta-oxidation intermediates on mitochondrial oxidative phosphorylation J. Inherit. Metab. Dis. 19 1996 161 164
31. W. Seubert, I. Lamberts, R. Kramer, and B. Ohly On the mechanism of malonyl-CoA-independent fatty acid synthesis. I. The mechanism of elongation of long-chain fatty acids by acetyl-CoA Biochim. Biophys. Acta 164 1968 98 517
32. P.K. Smith, R.I. Krohn, G.T. Hermanson, A.K. Mallia, F.H. Gartner, M.D. Provenzano, E.K. Fujimoto, N.M. Goeke, B.J. Olson, and D.C. Klenk Measurement of protein using bicinchoninic acid Anal. Biochem. 150 1985 76 85
33. P.A. Srere Citrate synthase Methods Enzymol. 13 1969 3 11
34. R.J. Wanders, J.P. Ruiter, and F.A. Wijburg Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: application to mitochondrial encephalomyopathies Biochim. Biophys. Acta 1181 1993 219 222
35. J.R. Williamson, and B.E. Corkey Assay of citric acid cycle intermediates and related compounds-update with tissue metabolite levels and intracellular distribution Methods Enzymol. 55 1979 200 222
36. R.W. Estabrook Mitochondrial respiratory control and the polarographic measurement of ADP:O ratios Methods Enzymol. 10 1967 41 47
37. J.B. Chappell, and K.M. Haardoff The penetration of the mitochondrial membrane by anions and cations E.C. Slater Z. Kaniuga L. Wojtczak Biochemistry of Mitochondria 1967 Academic Press/Pollish publishers London and New York/Warsaw 75 91
38. K.F. LaNoue, and A.C. Schoolwerth Metabolite transport in mitochondria Ann. Rev. Biochem. 48 1979 871 922
39. F. Morel, G. Lauquin, J. Lunardi, J. Duszynski, and P.V. Vignais An appraisal of the functional significance of the inhibitory effect of long chain acyl-CoAs on mitochondrial transports FEBS Lett. 39 1974 133 138
40. S.C. Halle-Smith, A.G. Murray, and M.J. Selwyn Palmitoyl-CoA inhibits the mitochondrial inner membrane anion-conducting channel FEBS Lett. 236 1988 155 158
41. G. Fiermonte, V. Dolce, R. Arrigoni, M.J. Runswick, J.E. Walker, and F. Palmieri Organization and sequence of the gene for the human mitochondrial dicarboxylate carrier: evolution of the carrier family Biochem. J. 344 1999 953 960
42. F.V. Ventura, J.P. Ruiter, L. IJlst, I.T. de Almeida, and R.J. Wanders Lactic acidosis in long-chain fatty acid beta-oxidation disorders J. Inherit. Metab. Dis. 21 1998 645 654