Menadione partially restores NADH-oxidation and ATP-synthesis in complex i deficient fibroblasts
Wijburg, F.A.; Feller, N.; de Groot, C.J.; Wanders, R.J.
Biochemistry International 22(2): 303-309
1990
ISSN/ISBN: 0158-5231 PMID: 2128595 Document Number: 362407
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.