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Menadione partially restores NADH-oxidation and ATP-synthesis in complex I deficient fibroblasts
F A Wijburg1, N Feller, C J de Groot
1Department of Pediatrics, University Hospital of Amsterdam (AMC), The Netherlands.
Abstract:
In this paper we report our studies on the effects of menadione in cultured fibroblasts treated with rotenone to block complex I. A normalization of the lactate to pyruvate ratio after incubation with glucose, an increased production of 14CO2 from [6-14C]glucose and an increased intra-cellular concentration of ATP was observed in the presence of micromolar concentrations of menadione. These results not only demonstrate the potential value of menadione in complex I deficient patients but also suggest that this system can be used advantageously for the in vitro assessment of therapeutic agents for disorders of the mitochondrial respiratory chain.