title: Simple models of threshold curves in the expression of inborn errors of metabolism: application to some experimental observations.
abstract: The expression of an enzymatic deficiency in a metabolic network can present a biochemical threshold. This threshold can be characterised thus: (1) a low activity of the enzyme can sustain a normal flux, but (2) a minute further decrease of its activity makes the flux collapse. We give simple mathematical models displaying such a behaviour, and we apply the models to some examples of oxidative phosphorylation dependency on respiratory chain complex deficiency.
authors: INSERM EMI 9929, Université Bordeaux 2, Bordeaux, France. jpm@u-bordeaux2.fr
authors: J P
authors: JP
authors: Mazat
authors: R
authors: R
authors: Rossignol
authors: M
authors: M
authors: Malgat
authors: T
authors: T
authors: Letellier
mesh_terms: D003198:Computer Simulation; D004579:Electron Transport / Q000502:physiology; D042967:Electron Transport Complex I; D006801:Humans; D007700:Kinetics; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism; D017240:Mitochondrial Myopathies; D008954:Models, Biological; D009247:NADH, NADPH Oxidoreductases / Q000378:metabolism; D010085:Oxidative Phosphorylation
chemical_list: D009247:NADH, NADPH Oxidoreductases; D042967:Electron Transport Complex I