To date, only few mutations in the mitochondrial DNA (mtDNA)-encoded ND2 subunit of Complex I have been reported, usually presenting a severe phenotype characterized by early onset encephalomyopathy and early death. In this report, we describe a new mutation in the MTND2 gene in a 21-year-old man with a mild myopathic phenotype characterized by exercise intolerance and increased plasma lactate ...

Despite advances in basic and clinical research, metastasis remains the leading cause of death in breast cancer patients. Genetic abnormalities in mitochondria, including mutations affecting complex I and oxidative phosphorylation, are found in breast cancers and might facilitate metastasis. Genes encoding complex I components have significant breast cancer prognostic value. In this study, we u...

In [ l ] low concentrations of Tinopal AN, (1,l-bis(3,N-5-dimethylbenzoxazol-2-y1)methine p-toluene sulphonate), which is present in the optical brightener Uvitex AN (Ciba-Geigy Ltd) [2], inhibited the aerobic respiration of Paracoccus denitrificans. Using inverted membrane vesicles of P. denitrifcans [I], this inhibition was shown to occur in the NADH dehydrogenase (EC 1.66.99.3) region of the...

Submitochondrial particles from bovine heart in which NADH dehydrogenase is reduced by either addition of NADH and rotenone or by reversed electron transfer generate 0.9 +/- 0.1 nmol of O2-/min per mg of protein at pH 7.4 and at 30 degrees C. When NADH is used as substrate, rotenone, antimycin and cyanide increase O2- production. In NADH- and antimycin-supplemented submitochondrial particles, r...

The activities of 4-aminobutyric-2-oxoglutaric acid transaminase (GABA-T) and succinate semialdehyde dehydrogenase (SSADH) were determined in mouse hypothalamus after peripheral injections of cholecystokinin-8 (CCK-X)and/or caerulein (CLN). GABA transaminase activity was measured utilizing endogenous succinate semialdellyde dehydrogenase to convert the product of GAB A-T, succinate semiald...

We developed and validated two species-specific qPCR markers to detect American Eel environmental DNA. Marker validation included assay design, specificity sensitivity testing, in vivo laboratory field experiments. Markers AME1 AME2 targeted 116 129 bp fragments of the mitochondrial NADH dehydrogenase subunit 2 cytochrome b genes, respectively. were 94%–100% homologous for all 49 aligned sequen...

Mutations in mitochondrial oxidative phosphorylation complex I are associated with multiple pathologies, and complex I has been proposed as a crucial regulator of animal longevity. In yeast, the single-subunit NADH dehydrogenase Ndi1 serves as a non-proton-translocating alternative enzyme that replaces complex I, bringing about the reoxidation of intramitochondrial NADH. We have created transge...

A gene encoding an NADH-dependent short-chain dehydrogenase/reductase (gox2036) from Gluconobacter oxydans 621H was cloned and heterogeneously expressed in Escherichia coli. The protein (Gox2036) was purified to homogeneity and biochemically characterized. Gox2036 was a homotetramer with a subunit size of approximately 28 kDa. Gox2036 had a strict requirement for NAD⁺/NADH as the cofactor. Gox2...

Chicken liver xanthine dehydrogenase can be partially reduced by either xanthine or NADH. Reduction to approximately the 2-electron-reduced level occurs with NADH, and reduction beyond the 2-electron level occurs with xanthine. In both cases, the reaction is triphasic. The first and third phases are dependent on reductant concentration, whereas the second phase is not. Oxidation of fully (6-ele...

This study investigated the effect of the anthracycline antibiotics on oxygen radical metabolism by cardiac mitochondrial reduced nicotinamide adenine dinucleotide (NADH) dehydrogenase [NADH:(acceptor) oxidoreductase, EC 1.6.99.3]. Superoxide formation by NADH dehydrogenase after anthracycline treatment appeared to follow saturation kinetics with an apparent Km of 167.3, 73.3, 64.0, or 47.6 mic...