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Article
Open AccessAuthor Correction: miR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophy
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Article
Open AccessmiR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophy
Duchenne Muscular Dystrophy (DMD) is a lethal muscle disorder, caused by mutations in the DMD gene and affects approximately 1:5000–6000 male births. In this report, we identified dysregulation of members of t...
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Article
Open AccessArabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls
Complex I (CI or NADH:ubiquinone oxidoreductase) deficiency is the most frequent cause of mitochondrial respiratory chain defect. Successful attempts to rescue CI function by introducing an exogenous NADH dehy...
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Article
Open AccessAcute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis
Mitochondria are key cellular signaling platforms, affecting fundamental processes such as cell proliferation, differentiation and death. However, it remains unclear how mitochondrial signaling affects other o...
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Article
Open AccessThe environmental carcinogen benzo[a]pyrene induces a Warburg-like metabolic reprogramming dependent on NHE1 and associated with cell survival
Cancer cells display alterations in many cellular processes. One core hallmark of cancer is the Warburg effect which is a glycolytic reprogramming that allows cells to survive and proliferate. Although the con...
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Article
Open AccessRapid determination of tricarboxylic acid cycle enzyme activities in biological samples
In the last ten years, deficiencies in tricarboxylic acid cycle (TCAC) enzymes have been shown to cause a wide spectrum of human diseases, including malignancies and neurological and cardiac diseases. A prereq...
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The Electron Transport Chain and Carcinogenesis
Major metabolic changes that affect the balance between respiration and glycolysis occur during carcinogenesis. It is therefore not surprising that it has long been suggested that abnormal activity of the mito...
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Article
Decylubiquinol impedes mitochondrial respiratory chain complex I activity
We have studied the interaction of decylubiquinone, an effective substrate for respiratory chain complexes III and II, with complex I in mouse and human tissues. We found that its reduced form, decylubiquinol,...
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Article
Genoty** microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt −1) in the NDUFS4 gene in Leigh syndrome
Complex I deficiency, the most common cause of mitochondrial disorders, accounts for a variety of clinical symptoms and its genetic heterogeneity makes identification of the disease genes particularly tedious....
