Additive Effects of Mitochondrion-targeted Cytochrome CYP2E1 and Alcohol Toxicity on Cytochrome c Oxidase Function and Stability of Respirosome Complexes
Seema BansalSatish SrinivasanSuresh Kumar AnandasadagopanAnindya Roy ChowdhuryVenkatesh SelvarajBalaraman KalyanaramanJoy JosephNarayan G. Avadhani
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Patients with mitochondrial respiratory-chain defects frequently exhibit lactic acidosis, ragged red fibers in skeletal muscle samples, and abnormal enzyme assays for the respiratory-chain complex. However, ragged red fibers and lactic acidosis are not always seen in all patients with mitochondrial respiratory-chain defects. We have encountered six children with biochemically proven respiratory chain defects, but typical ragged red fibers were not found in all six patients, and only five patients had increased serum lactate levels. Initially, they present with nonspecific features. However, persistent or progressive clinical features or multiple organ involvement eventually led to the diagnosis of respiratory-chain defects in these patients. Mitochondrial respiratory-chain defects should be considered in the differential diagnosis when persistent, progressive features and especially multiple organ involvement occur.
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To evaluate the effect of carabrone on mitochondrial respiratory chain complexes in Gaeumannomyces graminis to identify carabrone targets.The influence of carabrone on mitochondria in G. graminis mycelia was determined, and the activity of complexes I-V, citrate synthase, and respiratory chain complexes I+III and II+III was evaluated following treatment with 30% effective concentration (EC30 ) as well as EC50 and EC70 of carabrone for 0, 6, 12 and 24 h. Quantitative real-time PCR analysis of the relative expression of genes encoding mitochondrial respiratory chain complexes was then conducted. Carabrone treatment influenced the activity of mitochondrial respiratory chain complexes III, I+III, and II+III in G. graminis in a dose- and time-dependent manner with a marked decrease (c. 50% compared to the control) in mitochondrial respiratory chain complex III activity following treatment with carabrone at the EC50 . The GgCyc1 was downregulated following carabrone treatment at the EC50 , and the other genes were upregulated with respect to the control.This study showed that mitochondrial respiratory chain complex III in G. graminis is highly sensitive to carabrone and is a potential target of this botanical fungicidal agent.Carabrone is a promising botanical fungicidal agent that is environmentally friendly and can control plant diseases and reduce chemical fungicides in agricultural production.
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This article reviews the structure and function of mitochondrial respiratory chain complex Ⅱ, and the clinical features, diagnosis, treatment and genetic analysis of mitochondrial respiratory chain complex Ⅱ deficiency. Mitochondrial complex Ⅱ, known as succinate dehydrogenase, is a part of the mitochondrial respiratory chain. It plays an important role in cellular oxidative phosphorylation. It is associated with oxidative stress and is a sensitive target for toxic substances and abnormal metabolin in cells. Clinical manifestations of respiratory chain complex Ⅱ deficiency are characterized by a wide variety of abnormalities. Progressive neuromuscular dysfunction is the most common syndrome. Cardiomyopathy, episodic vomit and hemolytic uremic syndrome are also encountered in a few cases. A precise diagnosis is dependent on enzyme activities assay of respiratory chain complexes and genetic analysis. Complex Ⅱ activities decreased in affected tissues. Pathogenic mutations in SDHA gene and SDHAF1 gene encoding assembly factor have been found so far. Clinical treatment aims at improving the mitochondrial function.
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Coenzyme Q10 (CoQ10) is involved in ATP production through electron transfer in the mitochondrial respiratory chain complex. CoQ10 receives electrons from respiratory chain complex I and II to become the reduced form, and then transfers electrons at complex III to become the oxidized form. The redox state of CoQ10 has been reported to be a marker of the mitochondrial metabolic state, but to our knowledge, no reports have focused on the individual quantification of reduced and oxidized CoQ10 or the ratio of reduced to total CoQ10 (reduced/total CoQ10) in patients with mitochondrial diseases. We measured reduced and oxidized CoQ10 in skin fibroblasts from 24 mitochondrial disease patients, including 5 primary CoQ10 deficiency patients and 10 respiratory chain complex deficiency patients, and determined the reduced/total CoQ10 ratio. In primary CoQ10 deficiency patients, total CoQ10 levels were significantly decreased, however, the reduced/total CoQ10 ratio was not changed. On the other hand, in mitochondrial disease patients other than primary CoQ10 deficiency patients, total CoQ10 levels did not decrease. However, the reduced/total CoQ10 ratio in patients with respiratory chain complex IV and V deficiency was higher in comparison to those with respiratory chain complex I deficiency. Measurement of CoQ10 in fibroblasts proved useful for the diagnosis of primary CoQ10 deficiency. In addition, the reduced/total CoQ10 ratio may reflect the metabolic status of mitochondrial disease.
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