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.
Research Article|August 15 1994
The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach
Biochem J (1994) 302 (1): 171-174.
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T Letellier, R Heinrich, M Malgat, J P Mazat; The kinetic basis of threshold effects observed in mitochondrial diseases: a systemic approach. Biochem J 15 August 1994; 302 (1): 171–174. doi: https://doi.org/10.1042/bj3020171
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