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Electron Transport Complex III (Coenzyme Q-Cytochrome-c Reductase)

Subscribe to New Research on Electron Transport Complex III

A multisubunit enzyme complex that contains CYTOCHROME B GROUP; CYTOCHROME C1; and iron-sulfur centers. It catalyzes the oxidation of ubiquinol to UBIQUINONE, and transfers the electrons to CYTOCHROME C. In MITOCHONDRIA the redox reaction is coupled to the transport of PROTONS across the inner mitochondrial membrane.

Also Known As:

Coenzyme Q-Cytochrome-c Reductase; Complex III; Bacterial Electron Transport Complex III; Coenzyme QH2-Cytochrome-c Reductase; Core I Protein, UCCreductase; Core I Protein, Ubiquinol-Cytochrome c Reductase; Core II Protein, UCCreductase; Core II Protein, Ubiquinol-Cytochrome c Reductase; Cytochrome b-c2 Oxidoreductase; Cytochrome bc1; Dihydroubiquinone-Cytochrome-c Reductase; Mitochondrial Electron Transport Complex III; QH(2)-Cytochrome-c Reductase; QH(2)-Ferricytochrome-c Oxidoreductase; Ubihydroquinone-Cytochrome-c Reductase; Ubiquinol-Cytochrome c Reductase; Ubiquinone-Cytochrome b-c2 Oxidoreductase; Coenzyme Q Cytochrome c Reductase; Coenzyme QH2 Cytochrome c Reductase; Core I Protein, Ubiquinol Cytochrome c Reductase; Core II Protein, Ubiquinol Cytochrome c Reductase; Cytochrome b c2 Oxidoreductase; Dihydroubiquinone Cytochrome c Reductase; Reductase, Ubiquinol-Cytochrome c; Ubihydroquinone Cytochrome c Reductase; Ubiquinol Cytochrome c Reductase; Ubiquinone Cytochrome b c2 Oxidoreductase; Cytochrome bc1 Complex; Ubiquinol-Cytochrome-c Reductase; Ubiquinol-ferricytochrome-c oxidoreductase

Networked: 237 relevant articles (3 outcomes, 23 trials/studies)

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Bio-Agent Context: Research Results

Experts

1.	Lesnefsky, Edward J: 7 articles (09/2012 - 01/2002)
2.	Chandel, Navdeep S: 7 articles (01/2012 - 06/2007)
3.	Schumacker, Paul T: 6 articles (02/2013 - 03/2002)
4.	Hoppel, Charles L: 6 articles (06/2009 - 01/2002)
5.	Kwon, Ho Jeong: 4 articles (10/2014 - 04/2010)
6.	Jung, Hye Jin: 4 articles (10/2014 - 04/2010)
7.	Rustin, Pierre: 4 articles (08/2013 - 07/2003)
8.	Guzy, Robert D: 4 articles (02/2013 - 06/2005)
9.	Moraes, Carlos T: 4 articles (12/2012 - 04/2007)
10.	Fellman, Vineta: 4 articles (01/2012 - 11/2002)

Related Diseases

1.	Mitochondrial Diseases (Mitochondrial Disease) 04/10/2014 - "Importantly, inhibition of ATPIF1 ameliorates complex III blockade in primary hepatocytes, a cell type afflicted in severe mitochondrial disease. " 03/01/2008 - "Measurement of complex III activity is critical to the diagnosis of human mitochondrial disease and the study of mitochondrial pathobiology. " 01/01/2015 - "Complex III (CIII) deficiency is one of the least common oxidative phosphorylation defects associated to mitochondrial disease. " 12/01/2012 - "We have created two neuron-specific mouse models of mitochondrial electron transport chain deficiencies involving defects in complex III (CIII) or complex IV (CIV). " 01/01/2012 - "Liver is a target organ in many mitochondrial disorders, especially if the complex III assembly factor BCS1L is mutated. "
2.	Lactic Acidosis 12/01/2013 - "A homozygous mutation in LYRM7/MZM1L associated with early onset encephalopathy, lactic acidosis, and severe reduction of mitochondrial complex III activity." 07/01/2003 - "A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis." 05/01/1989 - "Fatal lactic acidosis in infancy with a defect of complex III of the respiratory chain." 10/01/1984 - "Lactic acidosis and mitochondrial myopathy associated with deficiency of several components of complex III of the respiratory chain." 12/01/2000 - "We have reinvestigated a young woman, originally reported by us in 1983, who presented with exercise intolerance and lactic acidosis associated with severe deficiency of complex III and who responded to therapy with menadione and ascorbate. "
3.	Mitochondrial Encephalomyopathies (Mitochondrial Encephalomyopathy) 02/01/2009 - "Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient." 12/01/2000 - "Mitochondrial encephalomyopathy and complex III deficiency associated with a stop-codon mutation in the cytochrome b gene." 01/01/1993 - "The levels of mitochondrial electron transport system proteins cytochrome c oxidase (COX) and complex III were measured in muscle fibers of patients with mitochondrial encephalomyopathy using quantitative immunoelectron microscopy. " 05/15/2009 - "We found that the PGC-1alpha and/or PGC-1beta expression improved mitochondrial respiration in cells harboring a complex III or IV deficiency as well as in transmitochondrial cybrids harboring mitochondrial encephalomyopathy lactic acidosis and stroke A3243G tRNA((Leu)UUR) gene mutation. " 08/01/2011 - "General conclusions are that: (i) disorders of glycogen metabolism are more likely to cause 'fetal disease' than defects of the respiratory chain; (ii) mitochondrial encephalomyopathies, especially those due to defects of the nuclear genome, are frequent causes of neonatal or infantile diseases, typically Leigh syndrome, but usually do not cause fetal distress; (iii) notable exceptions include mutations in the complex III assembly gene BCS1L resulting in the GRACILE syndrome (growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and early death), and defects of mitochondrial protein synthesis, which are the 'new frontier' in mitochondrial translational research."
4.	Lung Injury 04/01/2009 - "Participation of mitochondrial respiratory complex III in neutrophil activation and lung injury." 04/01/2009 - "These results indicate that inhibition of mitochondrial complex III diminishes Toll-like receptor 4-induced neutrophil activation through a mechanism dependent on H(2)O(2) generation and also reduces the severity of lung injury due to LPS exposure, a pathophysiologic process in which neutrophils play a major role." 04/01/2009 - "Administration of myxothiazol to mice resulted in diminished mitochondrial complex III activity in the lungs and decreased severity of LPS-induced lung injury. "
5.	Stroke (Strokes) 05/15/2009 - "We found that the PGC-1alpha and/or PGC-1beta expression improved mitochondrial respiration in cells harboring a complex III or IV deficiency as well as in transmitochondrial cybrids harboring mitochondrial encephalomyopathy lactic acidosis and stroke A3243G tRNA((Leu)UUR) gene mutation. "

Related Drugs and Biologics

1.	Toll-Like Receptors (Toll-Like Receptor)
2.	Leu Transfer RNA
3.	Electron Transport Complex III (Coenzyme Q-Cytochrome-c Reductase)
4.	Reactive Oxygen Species (Oxygen Radicals)
5.	Proteins (Proteins, Gene)
6.	Oxidoreductases
7.	coenzyme Q10 (CoQ10)
8.	oligomycin sensitivity-conferring protein (complex V)
9.	Carrier Proteins (Binding Protein)
10.	Cytochromes b (Cytochrome b)

Related Therapies and Procedures

1.	Cochlear Implantation
2.	Liver Transplantation
3.	Heterologous Transplantation (Xenotransplantation)