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. 1994 Aug 15;302 ( Pt 1)(Pt 1):171-4.
doi: 10.1042/bj3020171.

The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach

T Letellier  1 , R Heinrich, M Malgat, J P Mazat
Affiliations

The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach

T Letellier et al. Biochem J. .

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.

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