线粒体疾病中观察到的阈值效应的动力学基础：一种系统方法。
The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach.
作者信息
Letellier T, Heinrich R, Malgat M, Mazat J P
机构信息
Université Bordeaux II, France.
出版信息
Biochem J. 1994 Aug 15;302 ( Pt 1)(Pt 1):171-4. doi: 10.1042/bj3020171.
Abstract
Threshold effects in the expression of metabolic diseases have often been observed in mitochondrial pathologies, i.e. the clinical demonstration of the disease appears only when the activity of a step has been reduced to a rather low level. We show experimentally that an inhibition of cytochrome c oxidase activity by cyanide, simulating a defect in this step, leads to a decrease in mitochondrial respiration which then exhibits a threshold behaviour similar to that observed in mitochondrial diseases. We discuss this behaviour in terms of metabolic control theory and construct a mathematical model simulating this behaviour.
摘要
在线粒体病理学中经常观察到代谢疾病表达中的阈值效应，即只有当某一步骤的活性降低到相当低的水平时，疾病的临床症状才会显现出来。我们通过实验表明，用氰化物抑制细胞色素c氧化酶活性，模拟该步骤中的缺陷，会导致线粒体呼吸作用下降，进而表现出与线粒体疾病中观察到的类似的阈值行为。我们根据代谢控制理论讨论这种行为，并构建一个模拟这种行为的数学模型。
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