Methylmalonic Acid, a Biochemical Hallmark of Methylmalonic Acidurias but No Inhibitor of Mitochondrial Respiratory Chain

Journal of Biological Chemistry

Volumn 278, Issue 48, 2003, Pages 47388-47393

  Kölker, Stefan ^a   Schwab, Marina ^a   Hörster, Friederike ^a   Sauer, Sven ^a   Hinz, Angela ^a   Wolf, Nicole I ^a   Mayatepek, Ertan ^b   Hoffmann, Georg F ^a   Smeitink, Jan A M ^c   Okun, Jürgen G ^a  

^a UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

^b UNIVERSITY HOSPITAL DÜSSELDORF   (Germany)

^c RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

METABOLITES; NEUROLOGY; ORGANIC ACIDS; PATHOLOGY; RADIOMETRY; SPECTROPHOTOMETRY;

RENAL FAILURE;

BIOCHEMISTRY;

2 METHYLCITRIC ACID; ADENOSINE TRIPHOSPHATASE; ADENOSINE TRIPHOSPHATE; CARBON 14; CITRIC ACID; CYTOCHROME C OXIDASE; MALIC ACID; METHYLMALONIC ACID; PROPIONYL COENZYME A; PYRUVIC ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUCCINATE DEHYDROGENASE (UBIQUINONE); SUCCINIC ACID; UBIQUINOL CYTOCHROME C REDUCTASE; UNCLASSIFIED DRUG;

ANIMAL TISSUE; ARTICLE; CATTLE; CITRIC ACID CYCLE; ELECTRON TRANSPORT; ENERGY METABOLISM; ENZYME INHIBITION; FEMALE; MALE; METHYLMALONIC ACIDURIA; MOUSE; NERVE DEGENERATION; NONHUMAN; PRIORITY JOURNAL; RESPIRATORY CHAIN; SPECTROPHOTOMETRY;

ACYL COENZYME A; ADENOSINE TRIPHOSPHATE; AGING; AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; CATTLE; CITRATES; CITRIC ACID CYCLE; DOSE-RESPONSE RELATIONSHIP, DRUG; ELECTRON TRANSPORT; FEMALE; HYDROGEN-ION CONCENTRATION; MALE; MALONATES; METHYLMALONIC ACID; MICE; MICE, INBRED C57BL; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MUSCLES; MYOCARDIUM; OXYGEN; PHOSPHORYLATION; PYRUVATES; SPECTROPHOTOMETRY; TIME FACTORS;

ANIMALIA; BOS TAURUS; BOVINAE;

EID: 10744221377     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M308861200     Document Type: Article

References (35)

1. Fenton, W. A., Gravel, R. A., and Rosenblatt, D. S. (2001) in The Metabolic and Molecular Bases of inherited Disease (Scriver, C. R., Beaudet, A. L., Valle, A. D., and Sly, W. S., eds) pp. 2165-2193, McGraw-Hill Inc., New York
2. Mahoney, M. J., Hart, A. C., Stehen, V. D., and Rosenberg, L. E. (1975) Proc. Natl. Acad. Sci. U. S. A. 72, 2799-2803
3. Ledley, F. D., Lumetta, M. R., Zoghbi, H. Y., van Tuinen, P., Ledbetter, S. A., and Ledbetter, D. H. (1998) Am. J. Hum. Genet. 42, 839 -846
4. van der Meer, S. B., Poggi, F., Spada, M., Bonnefont, J. P., Ogier, H., Hubert, P., Depondt, E., Rapoport, D., Rabier, D., Charpentier, C., Parvy, P., Bardet, J., Kamoun, P., and Saudubray, J. M. (1994) J. Pediatr. 125, 903-908
5. Brismar, J., and Ozand, P. T. (1994) Am. J. Neuroradiol. 15, 1459 -1473
6. Larnaout, A., Mongalgi, M. A., Kaabachi, N., Khiari, D., Debbabi, A., Mebazza, A., Ben Hamida, M., and Hentati, F. (1998) J. Inherited Metab. Dis. 21, 639-644
7. Heidenreich, R., Natowicz, M., Hainline, B. E., Berman, P., Kelley, R. I., Hillman, R. E., and Berry, G. T. (1988) J. Pediatr. 113, 1022-1027
8. Trinh, B. C., Melhem, E. R., and Barker, P. B. (2001) Am. J. Neuroradiol. 22, 831-833
9. Martin, J. J., van de Vyver, F. L., Scholte, H. R., Roodhooft, A. M., Martin, C. C., and Luyt-Houwen, L. E. (1988) J. Neurol. Sci. 84, 189-200
10. Dutra, J. C., Dutra-Filho, C. S., Cardozo, S. E. C., Wannmacher, C. M. D., Sarkis, J. J. F., and Wajner, M. (1993) J. Inherited Metab. Dis. 16, 147-153
11. Brusque, A. M., Borba Rosa, R., Schluck, P. F., Dalcin, K. B., Ribeiro, C. A., Silva, C. G., Wannmacher, C. M., Dutra-Filho, C. S., Wyse, A. T., Briones, P., and Wajner, M. (2002) Neurochem. Int. 40, 593-601
12. Wajner, M., and Coelho, J. C. (1997) J. Inherited Metab. Dis. 20, 761-768
13. McLaughlin, B. A., Nelson, D., Silver, J. A., Erecinska, M., and Chesselet, M. F. (1998) Neuroscience 86, 276-290
14. Kölker, S., Ahlemeyer, B., Krieglstein, J., and Hoffmann, G. F. (2000) J. Inherited Metab. Dis. 23, 355-358
15. Okun, J. G., Hörster, F., Farkas, L. M., Feyh, P., Hinz, A., Sauer, S., Hoffmann, G. F., Unsicker, K., Mayatepek, E., and Kölker, S. (2002) J. Biol. Chem. 277, 14674-14680
16. de Mello, C. F., Begnini, J., Jimenez-Bernal, R. E., Rubin, M. A., de Bastiani, J., da Costa, E., Jr., and Wajner, M. (1996) Brain Res. 721, 120-125
17. Narasimhan, P., Sklar, R., Murrell, M., Swanson, R. A., and Sharp, F. R. (1996) J. Neurosci. 16, 7336-7346
18. Fighera, M. R., Queiroz, C. M., Stracke, M. P., Brauer, M. C., Gonzalez-Rodriquez, L. L., Frussa-Filho, R., Wajner, M., and de Mello, C. F. (1999) Neuroreport 10, 2039-2043
19. Albin, R. L., and Greenamyre, J. T. (1992) Neurology 42, 733-738
20. Okun, J. G., Lfimmen, P., and Brandt, U. (1999) J. Biol. Chem. 274, 2625-2630
21. Lowry, O. H., Roseborough, N. R., and Farr, A. J. (1951) J. Biol. Chem. 193, 265-275
22. Helenius, A., and Simons, K. (1972) J. Biol. Chem. 247, 3656-3661
23. Kölker, S., Okun, J. G., Hörster, F., Assmann, B., Kohlmüller, D., Exner-Camps, S., Ahlemeyer, B., Mayatepek, E., Krieglstein, J., and Hoffmann, G. F. (2001) J. Neurosci. Res. 66, 666-673
24. Beal, M. F., Brouillet, E., Jenkins, B. G., Ferrante, R. J., Kowall, N. W., Miller, J. M., Storey, E., Srivastava, R., Rosen, B. R., and Hyman, B. (1993) J. Neurosci. 13, 4181-4191
25. Brandt, U., and Okun, J. G. (1997) Biochemistry 36, 11234-11240
26. Fisher, J. C., Ruitenbeek, W., Stadhouders, A. M., Trijbels, J. M. F., Sengers, R. C. A., Janssen, A. J. M., and Veerkamp, J. H. (1985) Clin. Chim. Acta 145, 89-100
27. Trijbels, J. M. F., Sengers, R. C. A., Ruitenbeek, W., Fischer, J. C., Bakkeren, J., and Janssen, A. J. M. (1988) Eur. J. Pediatr. 148, 92-97
28. Sperl, W., Trijbels, J. M. F., Ruitenbeek, W., van Laack, H. L. J. M., Janssen, A. J. M., Kerkhof, C. M. C., and Sengers, R. C. A. (1993) Enzyme Protein 47, 37-46
29. Srere, P. A. (1969) in Methods in Enzymology (Löwenstein, J. M., eds) pp. 3-11, Academic Press, Inc., London
30. Ruitenbeek, W., Sengers, R. C. A., Trijbels, J. M. F., Stadhouders, A. M., and Janssen, A. J. M. (1981) J. Inherited Metab. Dis. 4, 91-92
31. Greene, J. G., Porter, R. H., Eller, R. V., and Greenamyre, J. T. (1993) J. Neurochem. 61, 1151-1154
32. Cheema-Dhadli, S., Leznoff, C. C., and Halperin, M. L. (1975) Pediatr. Res. 9, 905-908
33. Halperin, M. L., Schiller, C. M., and Fritz, I. B. (1971) J. Clin. Invest. 50, 2276-2282
34. Oberholzer, V. G., Levin, B., Burgess, E. A., and Young, W. F. (1967) Arch. Dis Child. 42, 492-504
35. Wyse, E. L, Streck, A. T., Barros, S. V., Brusque, A. M., Zugno, A. I., and Wajner, M. (2000) Neuroreport 11, 2331-2334