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.
作者： S. Krahenbuhl , M. Chang , E.P. Brass , C.L. Hoppel
DOI: 10.1016/S0021-9258(18)54810-9
关键词: Respiratory chain 、 Coenzyme Q – cytochrome c reductase 、 Biochemistry 、 Cytochrome 、 Oxidoreductase complex 、 Oxidoreductase 、 Hepatocyte 、 Ubiquinol 、 Biology 、 Mitochondrion
摘要: Rats treated with hydroxycobalamin[c-lactam] (HCCL), a cobalamin analogue that induces methylmalonic aciduria, have increased hepatic mitochondrial content and oxidative metabolism of pyruvate palmitate per hepatocyte. The present studies were undertaken to characterize in isolated liver mitochondria from rats HCCL. After 5-6 weeks, state 3 oxidation rates for diverse substrates are reduced HCCL-treated rats. Similar reductions obtained dinitrophenol-uncoupled excluding defective phosphorylation as cause the observed decrease oxidation. activities oxidases demonstrate defect complex IV. Investigation complexes respiratory chain reveals 32% ubiquinol:ferricytochrome c oxidoreductase (complex III) activity 72% ferrocytochrome c:oxygen IV) 5-6-week compared controls. Liver also decreased cytochrome mg protein (25% b 52% + a3 control rats). rat represents an animal model study consequences defects mitochondria.
sci-hub.st 参考文章(34)
E P Brass, S P Stabler, Carnitine metabolism in the vitamin B-12-deficient rat. Biochemical Journal. ,vol. 255, pp. 153- 159 ,(1988) , 10.1042/BJ2550153
Antonio Giuditta, Thomas P. Singer, Studies on succinic dehydrogenase. X. Reactivity with electron carriers in respiratory chain preparations from heart. Journal of Biological Chemistry. ,vol. 234, pp. 662- 665 ,(1959) , 10.1016/S0021-9258(18)70265-2
S P Stabler, E P Brass, R H Allen, L J Ruff, Effect of hydroxycobalamin[c-lactam] on propionate and carnitine metabolism in the rat. Biochemical Journal. ,vol. 266, pp. 809- 815 ,(1990)
P.A. Srere, [1] Citrate synthase Citric Acid Cycle. ,vol. 13, pp. 3- 11 ,(1969) , 10.1016/0076-6879(69)13005-0
David C. Wharton, Alexander Tzagoloff, [45] Cytochrome oxidase from beef heart mitochondria Methods in Enzymology. ,vol. 10, pp. 245- 250 ,(1967) , 10.1016/0076-6879(67)10048-7
Ronald W. Estabrook, [7] Mitochondrial respiratory control and the polarographic measurement of ADP : O ratios Methods in Enzymology. ,vol. 10, pp. 41- 47 ,(1967) , 10.1016/0076-6879(67)10010-4
Youssef Hatefi, Preparation and properties of NADH: ubiquinone oxidoreductase (complexI), EC 1.6.5.3. Methods in Enzymology. ,vol. 53, pp. 11- 14 ,(1978) , 10.1016/S0076-6879(78)53006-1
Youssef Hatefi, Diana L. Stiggall, [5] Preparation and properties of succinate: Ubiquinone oxidoreductase (complex II) Methods in Enzymology. ,vol. 53, pp. 21- 27 ,(1978) , 10.1016/S0076-6879(78)53008-5
Britton Chance, G.R. Williams, Respiratory enzymes in oxidative phosphorylation. IV. The respiratory chain. Journal of Biological Chemistry. ,vol. 217, pp. 429- 438 ,(1955) , 10.1016/S0021-9258(19)57192-7
Allan G. Gornall, Charles J. Bardawill, Maxima M. David, Determination of serum proteins by means of the biuret reaction. Journal of Biological Chemistry. ,vol. 177, pp. 751- 766 ,(1949) , 10.1016/S0021-9258(18)57021-6