Oxygen dependence of flux control of cytochrome c oxidase -: implications for mitochondrial diseases

被引：32

作者：

Wiedemann, FR ^{[1
]}

Kunz, WS ^{[1
]}

机构：

[1] Klinikum Otto von Guericke Univ Magdeburg, Neurol Klin, Neurobiochem Lab, D-39120 Magdeburg, Germany

来源：

FEBS LETTERS | 1998年 / 422卷 / 01期

关键词：

oxygen; cytochrome c oxidase; metabolic control analysis; mitochondrion; saponin-permeabilized fiber; mitochondrial disease;

D O I：

10.1016/S0014-5793(97)01586-X

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The oxygen dependence of cytochrome c oxidase control on succinate oxidation was investigated in saponin-permeabilized muscle fibers and isolated mitochondria from mouse quadriceps muscle applying metabolic control analysis. For this cyanide titrations of the oxygen consumption in the presence of succinate+rotenone were performed at different oxygen concentrations in the medium. While with isolated mitochondria high flux control coefficients were detected only at oxygen concentrations close to the K-M value of cytochrome c oxidase, with saponin-permeabilized fibers a significant increase of cytochrome c oxidase flux control was already observed below 130 mu M oxygen, The result is in line with the high oxygen sensitivity of maximal respiration of saponin-permeabilized muscle fibers (P-50 = 33 mu M) caused most probably by oxygen diffusion gradients through the fiber lattice, The oxygen dependence of cytochrome c oxidase flux control in muscle fibers can explain the pathological phenotype of mild cytochrome c oxidase deficiencies in mitochondrial myopathies. (C) 1998 Federation of European Biochemical Societies.

引用

页码：33 / 35

页数：3

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