Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1360, Issue 2, 1997, Pages 142-150

  Kuznetsov, Andrey V ^a   Winkler, Kirstin ^a   Kirches, Elmar ^a   Lins, Hartmut ^a   Feistner, Helmut ^a   Kunz, Wolfram S ^a  

^a OTTO VON GUERICKE UNIVERSITY   (Germany)

Author keywords

Flux control coefficient; Inhibitor titration; Metabolic control analysis; Mitochondrial disease; Saponin skinned muscle fiber

Indexed keywords

AZIDE; CYTOCHROME C OXIDASE; MITOCHONDRIAL DNA; OLIGOMYCIN; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATASE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); ROTENONE;

ADULT; ARTICLE; CONTROLLED STUDY; DELETION MUTANT; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME ACTIVITY; FEMALE; HUMAN; HUMAN TISSUE; IN VITRO STUDY; MALE; MITOCHONDRIAL RESPIRATION; MUSCLE BIOPSY; OXIDATIVE PHOSPHORYLATION; PRIORITY JOURNAL; QUANTITATIVE ASSAY; SKINNED MUSCLE FIBER; SOUTHERN BLOTTING; TECHNIQUE; THEORY; TITRIMETRY;

ADULT; AZIDES; BIOPSY; ELECTRON TRANSPORT COMPLEX I; ENZYME INHIBITORS; FEMALE; HUMANS; MALE; MITOCHONDRIAL MYOPATHIES; NADH, NADPH OXIDOREDUCTASES; OLIGOMYCINS; OXIDATIVE PHOSPHORYLATION; OXYGEN CONSUMPTION; PROTON-TRANSLOCATING ATPASES; ROTENONE;

EID: 0030891949     PISSN: 09254439     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0925-4439(96)00072-5     Document Type: Article

References (21)

1. Wallace, D.C. (1992) Annu. Rev. Biochem. 61, 1175-1212.
2. Morgan-Hughes, J.A. (1994) Curr. Opin. Neurol. 7, 457-462.
3. Robinson, B.H. (1993) Biochim. Biophys. Acta 1182, 231-244.
4. Kunz, W.S., Kuznetsov, A.V., Schulze, W., Eichhorn, K., Schild, L., Striggow, F., Bohnensack, R., Neuhof, S., Grasshoff, H., Neumann, H.W. and Gellerich, F.N. (1993) Biochim. Biophys. Acta 1144, 46-53.
5. Kunz, D., Luley, C., Kunz, W.S., Winkler, K., Fritz, S., Bohnensack, R. and Wallesch, C.-W. (1995) Biochem. Mol. Med. 54, 105-111.
6. Bentlage, H., de Coo, R., ter Laak, H., Sengers, R., Trijbels, F., Ruitenbeek, W., Schlote, W., Pfeiffer, K., Gencic, S., von Jagow, G. and Schägger, H. (1995) Eur. J. Biochem. 227, 909-915.
7. Kacser, H. and Burns, J.A. (1973) In Rate Control of Biological Processes (Davies, D.D. ed.), Cambridge University Press, London, pp. 65-104.
8. Heinrich, R. and Rapoport, T.A. (1974) Eur. J. Biochem. 42, 97-105.
9. Taylor, R.W., Birch-Machin, M.A., Bartlett, K., Lowerson, S.A. and Turnbull, D.M. (1994) J. Biol Chem. 269, 3523-3528.
10. Letellier, T., Malgat, M. and Mazat, J.P. (1993) Biochim. Biophys. Acta 1141, 58-64.
11. Schuster, R. and Holzhütter, H.-G. (1995) Eur. J. Biochem. 229, 403-418.
12. Kuznetsov, A.V., Clark, J.F., Winkler, K. and Kunz, W.S. (1996) J. Biol. Chem. 271, 283-288.
13. Moraes, C.T., Shanskke, S., Tritschler, H.-J., Aprille, J.R., Andreetta, F., Bonilla, E., Schon, E.A., DiMauro, S. (1991) Am. J. Hum. Gen. 48, 492-501.
14. Bergmeier, H.U. (1970) Methoden der enzymatischen Analyse. 2. Auflage. Akademie Verlag, Berlin, 1970.
15. Gellerich, F.N., Kunz, W.S. and Bohnensack, R. (1991) FEBS Lett. 274, 167-170.
16. Wisniewski, E., Kunz, W.S. and Gellerich, F.N. (1993) J. Biol. Chem. 268, 9343-9346.
17. Wisniewski, E., Gellerich, F.N. and Kunz, W.S. (1995) Eur. J. Biochem. 230, 549-554.
18. Li, W. and Palmer, G. (1993) Biochemistry 32, 1833-1843.
19. Bentlage, H.A.C.M., Janssen, A.J.M., Chomyn, A., Attardi, G., Walker, J.E., Schägger, H., Sengers, R.C.A. and Trijbels, F.J.M. (1995) Biochim. Biophys. Acta 1234, 63-73.
20. Winkler, K., Kuznetsov, A.V., Lins, H., Kirches, E., von Bossanyi, P., Dietzmann, K., Frank, B., Feistner, H. and Kunz, W.S. (1995) Biochim. Biophys. Acta 1272, 181-184.
21. Letellier, T., Heinrich, R., Malgat, M. and Mazat, J.-P. (1994) Biochem. J. 302, 171-174.