CONTROL OF OXIDATIVE-PHOSPHORYLATION IN RAT MUSCLE MITOCHONDRIA - IMPLICATIONS FOR MITOCHONDRIAL MYOPATHIES

被引：103

作者：

LETELLIER, T ^{[1
]}

MALGAT, M ^{[1
]}

MAZAT, JP ^{[1
]}

机构：

[1] UNIV BORDEAUX 2, 146 RUE LEO SAIGNAT, F-33076 BORDEAUX, FRANCE

来源：

BIOCHIMICA ET BIOPHYSICA ACTA | 1993年 / 1141卷 / 01期

关键词：

METABOLISM CONTROL; MUSCLE MITOCHONDRION; MYOPATHY; OXIDATIVE PHOSPHORYLATION; MITOCHONDRIAL MYOPATHY;

D O I：

10.1016/0005-2728(93)90189-M

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 control of oxidative phosphorylation has been studied in normal skeletal muscle isolated from the hind legs of rats. The control coefficients of different steps of oxidative phosphorylation on the flux of O2 consumption were determined by the inhibitor method and calculation was done according to the model of Gellerich et al. (FEBS Lett. 274 (1990) 167-170) using a non-linear regression fitting procedure. The respiration was recorded with pyruvate (+malate) and palmitoyl-L-carnitine (+malate) as respiratory substrates, which are the main substrates oxidized in the muscle. It appears that the control is broadly distributed among the different complexes of the respiratory chain, and of the ATP synthesis system. Our results also provide an explanation for the threshold effects often evidenced in the clinical manifestation of mitochondrial diseases.

引用

页码：58 / 64

页数：7

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