Published in Biochemistry on September 16, 1997
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The Q-cycle reviewed: How well does a monomeric mechanism of the bc(1) complex account for the function of a dimeric complex? Biochim Biophys Acta (2008) 1.43
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Modifications of protein environment of the [2Fe-2S] cluster of the bc1 complex: effects on the biophysical properties of the rieske iron-sulfur protein and on the kinetics of the complex. J Biol Chem (2009) 0.95
Identification of ubiquinol binding motifs at the Qo-site of the cytochrome bc1 complex. J Phys Chem B (2014) 0.90
The mechanism of ubihydroquinone oxidation at the Qo-site of the cytochrome bc1 complex. Biochim Biophys Acta (2013) 0.89
Electronic connection between the quinone and cytochrome C redox pools and its role in regulation of mitochondrial electron transport and redox signaling. Physiol Rev (2015) 0.86
Analysis of the kinetics and bistability of ubiquinol:cytochrome c oxidoreductase. Biophys J (2013) 0.85
Coenzyme Q(10) is decreased in fibroblasts of patients with methylmalonic aciduria but not in mevalonic aciduria. J Inherit Metab Dis (2009) 0.84
Stigmatellin induces reduction of iron-sulfur protein in the oxidized cytochrome bc1 complex. J Biol Chem (2008) 0.80
Role of the -PEWY-glutamate in catalysis at the Q(o)-site of the Cyt bc(1) complex. Biochim Biophys Acta (2012) 0.79
Effect of pH on the steady state kinetics of bovine heart NADH: coenzyme Q oxidoreductase. J Bioenerg Biomembr (2003) 0.79
Distinct properties of semiquinone species detected at the ubiquinol oxidation Qo site of cytochrome bc1 and their mechanistic implications. J R Soc Interface (2016) 0.77
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Guideline for the diagnosis and management of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I). J Inherit Metab Dis (2007) 1.31
Neurodegeneration and chronic renal failure in methylmalonic aciduria--a pathophysiological approach. J Inherit Metab Dis (2007) 1.22
Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome. Acta Anaesthesiol Scand (2012) 1.11
Enzymatic and metabolic evidence for a region specific mitochondrial dysfunction in brains of murine succinic semialdehyde dehydrogenase deficiency (Aldh5a1-/- mice). Neurochem Int (2007) 1.08
A single external enzyme confers alternative NADH:ubiquinone oxidoreductase activity in Yarrowia lipolytica. J Cell Sci (1999) 0.99
Maintenance treatment of glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis (2004) 0.93
Pathogenesis of CNS involvement in disorders of amino and organic acid metabolism. J Inherit Metab Dis (2008) 0.92
Excitotoxicity and bioenergetics in glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis (2004) 0.92
Three molecules of ubiquinone bind specifically to mitochondrial cytochrome bc1 complex. J Biol Chem (2001) 0.91
Abnormal sterol metabolism in holoprosencephaly: studies in cultured lymphoblasts. J Med Genet (2007) 0.90
3-Ureidopropionate contributes to the neuropathology of 3-ureidopropionase deficiency and severe propionic aciduria: a hypothesis. J Neurosci Res (2001) 0.86
Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen. Neuropediatrics (2003) 0.85
Coenzyme Q(10) is decreased in fibroblasts of patients with methylmalonic aciduria but not in mevalonic aciduria. J Inherit Metab Dis (2009) 0.84
Massive insulin secretion in response to anaerobic exercise in exercise-induced hyperinsulinism. Horm Metab Res (2005) 0.83
Methylmalonic acid--an endogenous toxin? Cell Mol Life Sci (2005) 0.82
Measurement of bile acid CoA esters by high-performance liquid chromatography-electrospray ionisation tandem mass spectrometry (HPLC-ESI-MS/MS). J Mass Spectrom (2005) 0.82
Animal models for glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis (2004) 0.79
Looking forward--an evidence-based approach to glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis (2004) 0.76
Long-term exposure of human proximal tubule cells to hydroxycobalamin[c-lactam] as a possible model to study renal disease in methylmalonic acidurias. J Inherit Metab Dis (2009) 0.76
Challenges for basic research in glutaryl-CoA dehydrogenase deficiency. J Inherit Metab Dis (2004) 0.75