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大鼠肌肉线粒体中氧化磷酸化的调控:对线粒体肌病的影响。

Control of oxidative phosphorylation in rat muscle mitochondria: implications for mitochondrial myopathies.

作者信息

Letellier T, Malgat M, Mazat J P

机构信息

Université Bordeaux II, France.

出版信息

Biochim Biophys Acta. 1993 Feb 8;1141(1):58-64. doi: 10.1016/0005-2728(93)90189-m.

DOI:10.1016/0005-2728(93)90189-m
PMID:8382080
Abstract

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.

摘要

对从大鼠后腿分离出的正常骨骼肌中的氧化磷酸化调控进行了研究。采用抑制剂法测定了氧化磷酸化不同步骤对氧气消耗通量的控制系数,并根据Gellerich等人(《欧洲生物化学学会联合会快报》274 (1990) 167 - 170)的模型,使用非线性回归拟合程序进行计算。以丙酮酸(+苹果酸)和棕榈酰-L-肉碱(+苹果酸)作为呼吸底物记录呼吸情况,这两种底物是肌肉中氧化的主要底物。结果表明,呼吸链和ATP合成系统的不同复合体之间广泛存在调控作用。我们的研究结果还为线粒体疾病临床表现中经常出现的阈值效应提供了解释。

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