2128595 |
Wijburg FA, Feller N, de Groot CJ, Wanders RJ: Menadione partially restores NADH-oxidation and ATP-synthesis in complex I deficient fibroblasts. Neurosci Lett. 2003 Nov 6;351(1):29-32. 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. |
82(1,1,1,2) |