• There are no respiratory dehydrogenases other than NDH-2 to feed electrons into the respiratory chain, and there is only one respiratory oxygen reductase to reduce oxygen to water. (uea.ac.uk)
  • Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. (nih.gov)
  • It transfers electrons from NADH to the respiratory chain. (wikipedia.org)
  • Initially, NADH binds to Complex I and transfers two electrons to the isoalloxazine ring of the flavin mononucleotide (FMN) prosthetic arm to form FMNH2. (wikipedia.org)
  • The flow of electrons changes the redox state of the protein, resulting in a conformational change and pK shift of the ionizable side chain, which pumps four hydrogen ions out of the mitochondrial matrix. (wikipedia.org)
  • Reducing equivalents (NADH, FADH 2 ) are generated by reactions catalysed by the PDC and the tricarboxylic acid cycle and donate electrons (e - ) that enter the respiratory chain at NADH ubiquinone oxidoreductase (complex I) or at succinate ubiquinone oxidoreductase (complex II). (bmj.com)
  • It pumps four protons through four transiently open pathways from the high pH, negative, N- side of the membrane to the positive, P-side driven by the exergonic transfer of electrons from NADH to a quinone. (cuny.edu)
  • The Krebs cycle produces NADH, which delivers electrons to complex I, and NADPH for H\(_2\)O\(_2\) elimination via isocitrate dehydrogenase and nicotinamide nucleotide transhydrogenase (NNT). (uni-wuerzburg.de)
  • Complex I functions in the transfer of electrons from NADH to the respiratory chain. (nih.gov)
  • It plays an important role in transfering electrons from NADH to the respiratory chain. (nih.gov)
  • This research project focused on how the movement of electrons from NADH to the Q site leads to formation of quinol (QH2), and how this chemical process triggers proton pumping across the membrane (see Fig. 1). (gauss-centre.eu)
  • A process in which a series of electron carriers operate together to transfer electrons from donors such as NADH and FADH2 to any of several different terminal electron acceptors to generate a transmembrane electrochemical gradient. (cathdb.info)
  • The main function of this enzyme is to oxidize excess of NADPH, forming NADH that supplies electrons to the respiratory chain. (expasy.org)
  • The electron transport chain ETC is a series of protein complexes that transfer electrons from electron donors to electron acceptors via redox reactions both reduction and oxidation occurring simultaneously and couples this electron transfer with the transfer of protons. (vumc.org)
  • Electrons are passed from one member of the transport chain to another in a series of redox reactions. (vumc.org)
  • B the thermodynamically favorable flow of electrons from NADH to the mitochondrial electron transport carriers. (vumc.org)
  • The electron transport chain is a series of molecules that accept or donate electrons easily. (vumc.org)
  • TgDHODH is localized in the inner mitochondrial membrane, and is linking the pyrimidine de novo pathway to the mitochondrial respiratory chain by transferring electrons from dihydroorotate to ubiquinone. (uni-goettingen.de)
  • This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). (nih.gov)
  • This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which is the first enzyme in the electron transport chain of mitochondria. (nih.gov)
  • NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NADH-ubiquinone oxidoreductase ESSS subunit) is an enzyme that in humans is encoded by the NDUFB11 gene. (wikipedia.org)
  • NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. (wikipedia.org)
  • NDUFB11 is a subunit of the enzyme NADH dehydrogenase (ubiquinone), the largest of the respiratory complexes. (wikipedia.org)
  • The protein encoded by this gene is an accessory subunit of the multisubunit NADH:ubiquinone oxidoreductase (complex I) that is not directly involved in catalysis. (wikipedia.org)
  • Strickland M, Juárez O, Neehaul Y, Cook DA, Barquera B, Hellwig P. (2014) The conformational changes induced by ubiquinone binding in the Na+-pumping NADH:ubiquinone oxidoreductase (Na+-NQR) are kinetically controlled by conserved glycines 140 and 141 of the NqrB subunit. (iit.edu)
  • Shea ME, Juárez O, Cho J, Barquera B. (2013) Aspartic acid 397 in subunit B of the Na+-pumping NADH:quinone oxidoreductase from Vibrio cholerae forms part of a sodium-binding site, is involved in cation selectivity, and affects cation-binding site cooperativity. (iit.edu)
  • Juárez O, Neehaul Y, Turk E, Chaubon N, Hellwig P and Barquera B. (2012) The role of Glycine residues 140 and 141 of NqrB subunit in the ubiquinone binding site of the sodium -dependent NADH dehydrogenase. (iit.edu)
  • Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. (nih.gov)
  • It interacts directly with CARBONIC ANHYDRASE 2 (CA2), an essential subunit of respiratory chain NADH dehydrogenase complex (Complex I) and regulates Complex I activity via redox modification of CA2. (utoronto.ca)
  • Remarkably, it is shown here that the entire respiratory chain of S. agalactiae consists of only two enzymes, a type 2 NADH dehydrogenase (NDH-2) and a cytochrome bd oxygen reductase. (uea.ac.uk)
  • Although bacteria usually have a branched respiratory chain with multiple dehydrogenases and terminal oxygen reductases, here we establish that S. agalactiae utilizes only one type 2 NADH dehydrogenase (NDH-2) and one cytochrome bd oxygen reductase to perform respiration. (uea.ac.uk)
  • Only those particles which contain 'turnover-preconditioned" NADH-ubiquinone reductase demonstrate the ubisemiquinone signal together with N-1, N-2, N-3 and N-4 iron-sulfur centers. (tau.ac.il)
  • Experiments with tritiated NADH are described which demonstrate that this "external" pathway of NADH oxidation resembles stereochemically the NADH-cytochrome c reductase system of liver microsomes, and differs from the respiratory chain-linked NADH dehydrogenase. (rupress.org)
  • Enzyme distributation data are presented which substantiate the conclusion that microsomal contamination cannot account for the rotenone-insensitive NADH-cytochrome c reductase activity observed with the mitochondria. (rupress.org)
  • A procedure is developed, based on swelling and shrinking of the mitochondria followed by sonication and density gradient centrifugation, which permits the separation of two particulate subfractions, one containing the bulk of the respiratory chain components, and the other the bulk of the rotenone-insensitive NADH-cytochrome c reductase system. (rupress.org)
  • The data indicate that the electron-transport system associated with the mitochondrial outer membrane involves catalytic components similar to, or identical with, the microsomal NADH-cytochrome b 5 reductase and cytochrome b 5 . (rupress.org)
  • Na(+)-translocating NADH-quinone reductase of marine and halophilic bacteria. (shengsci.com)
  • The respiratory chain of marine and moderately halophilic bacteria requires Na+ for maximum activity, and the site of Na(+)-dependent activation is located in the NADH-quinone reductase segment. (shengsci.com)
  • The Na(+)-dependent NADH-quinone reductase purified from marine bacterium Vibrio alginolyticus is composed of three subunits. (shengsci.com)
  • Deactivation of Complex I results in almost complete loss of its NADH-ubiquinone reductase activity and in increase in NADH-dependent superoxide generation. (scienceopen.com)
  • One site (F) serves as an entry for the NADH oxidation and the other one (R) serves as an exit during either the succinate-supported NAD+ reduction or superoxide generation or NADH-ferricyanide reductase reaction. (scienceopen.com)
  • Mamoru Yamada The respiratory chain of the ethanologenic bacterium Zymomonas mobilis was investigated, in which the pyruvate-to-ethanol pathway has been demonstrated to be mainly responsible for NADH oxidation. (karger.com)
  • These organelles are directly involved in the oxidation of acetyl-CoA in the Krebs cycle, leading to the formation of NADH and two CO 2 molecules. (assignmentexpert.com)
  • Preparations of rat-liver mitochondria catalyze the oxidation of exogenous NADH by added cytochrome c or ferricyanide by a reaction that is insensitive to the respiratory chain inhibitors, antimycin A, amytal, and rotenone, and is not coupled to phosphorylation. (rupress.org)
  • Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits. (nih.gov)
  • cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed. (nih.gov)
  • Isolated complex I deficiency is a rare inborn error of metabolism due to mutations in nuclear or mitochondrial genes encoding subunits or assembly factors of the human mitochondrial complex I (NADH: ubiquinone oxidoreductase) and is characterized by a wide range of manifestations including marked and often fatal lactic acidosis, cardiomyopathy, leukoencephalopathy, pure myopathy and hepatopathy with tubulopathy. (orpha.net)
  • Complex I, NADH-ubiquinone oxidoreductase, is the first enzyme in the mitochondrial and bacterial aerobic respiratory chain. (cuny.edu)
  • NADH:ubiquinone oxidoreductase, complex I, is one of the largest membrane protein assemblies known and has a central role in energy production by the mitochondrial respiratory chain, providing about 40 percent of the proton motive force required for the synthesis of adenosine triphosphate (ATP) that is the energy storage form of the cells. (mpg.de)
  • Reyes-Prieto A, Barquera B, Juárez O. (2014) Origin and evolution of the sodium -pumping NADH: ubiquinone oxidoreductase. (iit.edu)
  • Figure 1 shows the largest of those enzymes, complex I or NADH:ubiquinone oxidoreductase, which initiates the cell respiration process in many aerobic organisms. (gauss-centre.eu)
  • Generation of superoxide-radical by the NADH:ubiquinone oxidoreductase of heart mitochondria. (scienceopen.com)
  • The complexes studied are Complex I (NADH:Ubiquinone oxidoreductase), Complex II (Succinate:ubiquinone oxidoreductase), Complex III (Ubiquinol:cytochrome c oxidoreductase), and Complex IV (Cytochrome c oxidase). (cdc.gov)
  • The efficiency of the particles in exhibiting oxidative phosphorylation (P/O ratio) and NADH-linked NADP+ reduction (NADP+/O ratio) was measured, using succinate as oxidizable substrate and rotenone as inhibitor of Nadh oxidase, under conditions where the phosphorylating and transhydrogenating systems were operating one by one or in parallel. (eurekamag.com)
  • The electron transport chain is also called the Cytochrome oxidase system or as the Respiratory chain. (vumc.org)
  • In contrast to NADH oxidase, the rate-substrate concentration dependence for the superoxide production shows a maximum at low (approximately 50 microM) concentrations of NADH. (scienceopen.com)
  • A Na -dependent, 2-heptyl-4-hydroxyquinoline N -oxide (HQNO) supersensitive NADH oxidase was not present in strain 184. (microbiologyresearch.org)
  • Coupled bovine heart submitochondrial particles exhibit a rotenone-sensitive g = 2.00 low-temperature EPR signal attributable to ubisemiquinone which is observed during steady-state electron transfer from NADH to oxygen or from succinate to NAD + in Δ \ ̃gmH + -dependent reverse electron transfer. (tau.ac.il)
  • The intensity of the signal is drastically reduced when electron transfer from NADH to oxygen is blocked by cyanide or in the case of the reverse electron transfer from succinate to NAD + being prevented by anaerobiosis. (tau.ac.il)
  • NAD+ and NADH inhibit the succinate-supported superoxide generation. (scienceopen.com)
  • The effect of various electron donors (NADH, succinate and 2,3-dimethoxy-5-methyl-6-decyl- 1,4-benzoquinol ) on ROS production was tested separately in the presence or absence of Cd. (cdc.gov)
  • Next, the mitochondrial inner membrane contains proteins that are the components of the respiratory chain providing the transfer of electron and protons from NADH to oxygen resulting in the formation of H 2 O. Finally, electron transport chain localized in the mitochondrial inner membrane provides the formation of ATP associated with the activity of the ATP-synthetase complex. (assignmentexpert.com)
  • The energy conversion by these respiratory enzymes is achieved by pumping protons across a biological membrane. (gauss-centre.eu)
  • Since energy is required to move protons across the membrane, the enzymes of the respiratory chain use a series of exergonic chemical reactions and couple them to the proton translocation. (gauss-centre.eu)
  • Complex I is the first enzyme of the respiratory chain and it employs the energy from the electron transfer (eT) process from NADH to the quinone (Q) to pump four protons (H+) across the membrane. (gauss-centre.eu)
  • This pathogen respires aerobically if heme and quinone are available in the environment, and a functional respiratory chain is required for full virulence. (uea.ac.uk)
  • This organism can aerobically respire, but only using external sources of heme and quinone, required to have a functional electron transport chain. (uea.ac.uk)
  • Complex I, or NADH:quinone oxidoreductase, catalyzes the first step in the respiratory chain. (lu.se)
  • Complex I is the first enzyme of the respiratory chain and has a central role in cellular energy production, coupling electron transfer between NADH and quinone to proton translocation by an unknown mechanism. (mpg.de)
  • Neehaul Y, Juárez O, Barquera B, Hellwig P. (2013) IR spectroscopic evidence for a redox -dependent conformational change involving ion binding residue NqrB-D397 in the Na+ pumping NADH:quinone oxidoreductase from Vibrio cholerae. (iit.edu)
  • Juárez O, Barquera B. (2012) Insights into the mechanism of electron transfer and sodium translocation of the Na+-pumping NADH:quinone oxidoreductase. (iit.edu)
  • Respiratory complex I is the largest and most intricate enzyme of the respiratory chain and responsible for converting energy from the reduction of quinone into an electrochemical proton gradient. (gauss-centre.eu)
  • Nach einer Übersicht über das Wesen der NADH- und NADPH-Tetrazoliumreduktase, die früher auch als Diaphorasen bezeichnet wurden, wird über das Vorkommen dieser Enzyme und ihre Histotopie in normaler menschlicher Haut berichtet. (springer.com)
  • Dem Reaktionsausfall nach zeigt die NADH-Tetrazoliumreduktase in der Regel eine größere Aktivität als die NADPH-Tetrazoliumreduktase. (springer.com)
  • Alle lebenden epithelialen Strukturen der Haut sind durch eine histochemisch faßbare NADH- und NADPH-Tetrazoliumreduktaseaktivität ausgezeichnet. (springer.com)
  • In der Gesamtheit lassen die Untersuchungsergebnisse vermuten, daß - vielleicht mit Ausnahme des infraseboglandulären Haarfollikels -alle übrigen epithelialen Zellen durch eine so intensive NADH- und NADPH-Tetrazoliumreduktaseaktivität charakterisiert sind, daß diese wahrscheinlich für den Nachweis von Dehydrogenasen, soweit er unter Verwendung von Tetrazoliumsalzen geführt wird, einen reaktionsbegrenzenden Faktor nicht darstellen. (springer.com)
  • In conclusion, NADH from α-KGDH selectively shuttles to NNT for NADPH formation rather than to complex I of the respiratory chain for ATP production. (uni-wuerzburg.de)
  • The light energy is used by photosystems I and II to generate proton motive force and reducing power (NADPH or NADH). (kegg.jp)
  • The NADH binding site, FMN and eight FeS clusters are located in the promontory part, whereas the seven membrane-spanning proteins are all encoded by mitochondrial DNA in eukaryotes, in fact the Complex I encoding genes make up half of the mtDNA in mammals. (lu.se)
  • The electron transport chain is a series of proteins and organic molecules found in the inner membrane of the mitochondria. (vumc.org)
  • Usually, carbohydrates are oxidised to release energy but proteins, fats and even organic acids can be used as respiratory substances in some plants, under certain conditions. (cleariitmedical.com)
  • In heart failure, a functional block of complex I of the respiratory chain provokes superoxide generation, which is transformed to H\(_2\)O\(_2\) by dismutation. (uni-wuerzburg.de)
  • New genes encoding subunits of a cytochrome bc1-analogous complex in the respiratory chain of the hyperthermoacidophilic crenarchaeon Sulfolobus acidocaldarius. (shengsci.com)
  • Mitchell, P. Protonmotive redox mechanism of the cytochrome b - c 1 complex in the respiratory chain: protonmotive ubiquinone cycle. (nature.com)
  • Most identifiable cases involve inherited or spontaneous mutations in the pyruvate dehydrogenase complex (PDC) or in one or more enzymes of the respiratory chain. (bmj.com)
  • PDH kinase which are responsible for the inactive PDH is promoted by ATP, NADH and acetyl coenzyme A. while PDH complex is inhibited by NAD+, coenzyme A and pyruvate. (biotechfront.com)
  • Increases in the intramitochondrial ratios of NADH/NAD and acetyl CoA/CoA also stimulate kinase mediated phosphorylation of PDC. (bmj.com)
  • NADH-dependent respiration plays a critical role in the pathogen in maintaining NADH/NAD+ redox balance in the cell, optimizing ATP production, and tolerating oxygen. (uea.ac.uk)
  • The structure is L-shaped with a long, hydrophobic transmembrane domain and a hydrophilic domain for the peripheral arm that includes all the known redox centers and the NADH binding site. (wikipedia.org)
  • This protein complex has NADH dehydrogenase activity and oxidoreductase activity. (wikipedia.org)
  • This protein has NADH dehydrogenase activity and oxidoreductase activity. (nih.gov)
  • Northwestern Medicine investigators recently discovered that the mitochondrial respiratory chain-a series of protein complexes essential for a cellular respiration and energy production-is necessary for the activation of another protein complex linked to inflammation and the progression of chronic diseases, according to a study published in Nature Immunology . (medicalxpress.com)
  • Crystal structure of mitochondrial respiratory membrane protein complex II. (nature.com)
  • The deficient activity of long-chain 3-hydroxy acyl-coenzyme A dehydrogenase may be diagnosed in fibroblasts, as well as the other enzyme activities of the trifunctional protein. (medscape.com)
  • Finally, the reduced flavoprotein is oxidised by NAD and transfers reducing equivalents to the respiratory chain via NADH. (bmj.com)
  • FADH2 in turn transfers the reducing equivalents to NAD+ to give NADH and H+. (biotechfront.com)
  • The enzyme usually is measured in fibroblasts in the reverse direction, with 3-oxopalmitoyl CoA as substrate and measurement of the decrease in absorbance at 340 nm of the nicotinamide adenine dinucleotide-reduced form (NADH) electron donor. (medscape.com)
  • Furthermore, inhibitors of the mitochondrial respiratory chain complexes I, II, III and V-all of which are essential for proper production of energy within the cell-prevented NLRP3 inflammasome activation. (medicalxpress.com)
  • They also found that creatine, which is naturally produced by the body and is a widely available nutritional supplement, boosts levels of phosphocreatine, which is essential for proper energy production within cells, and is depleted by the respiratory chain inhibitors, which in turn also sustains activation of the inflammasome. (medicalxpress.com)
  • The proton motive force is used by ATP synthase to generate ATP, essentially in the same way as the mitochondrial respiratory chain. (kegg.jp)
  • Mitochondrial complex I deficiency is a disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to adult-onset neurodegenerative disorders. (wikipedia.org)
  • Net result is that in the presence of high-energy molecules like ATP, NADH. (biotechfront.com)
  • Respiratory substrates are compounds that are oxidised during the process of respiration. (cleariitmedical.com)
  • Tuz K, Mezic KG, Xu T, Barquera B, Juarez O (2016) The kinetic reaction mechanism of the Vibrio cholerae sodium-dependent NADH dehydrogenase. (iit.edu)
  • Diagnostic polymerase chain reaction (PCR) restriction fragment length polymor- stages (length of worms, number of phism analysis for Echinococcus multilocularis (lanes 1-8, 2 specimens in parallel) and E. proglottids, terminal proglottids in granulosus (lanes 9-12, 1 specimen). (cdc.gov)
  • Biochemically, there may be profound lactic acidosis because the NADH:NAD ratio increases, shifting the equilibrium of the lactate dehydrogenase reaction toward lactate. (msdmanuals.com)
  • It provides solid evidence that respiratory supercomplex formation in the fruit fly does not impact respiratory function, suggesting the role of these complexes is structural, rather than catalytic. (elifesciences.org)
  • Tuz K, Li C, Fang X, Raba DA, Liang P, Minh DD, Juarez O (2017) Identification of the catalytic ubiquinone-binding site of Vibrio cholerae sodium-dependent NADH dehydrogenase: a novel ubiquinone-binding motif. (iit.edu)
  • Uniparental isodisomy as a cause of mitochondrial complex I respiratory chain disorder due to a novel splicing NDUFS4 mutation. (nih.gov)
  • respiratory burst (the rapid release of Reactions of oxygen radicals with Mitochondrial oxidative phosphor- reactive oxygen species from cells) cel ular components can deplete an- ylation is a major source of oxy- that involves nicotinamide adenine tioxidants, can cause direct oxidative gen radicals of endogenous origin. (who.int)
  • It has the ability to catalyze several different enzymatic reactions in electron transport, and is known to be one of the respiratory chain components most sensitive to ischaemia. (karger.com)
  • Mammalian mitochondrial respiratory chain (MRC) complexes are able to associate into quaternary structures named supercomplexes (SCs), which normally coexist with non-bound individual complexes. (elifesciences.org)
  • We investigated the effects of Cd on the individual complexes of the electron transfer chain (ETC) and on stimulation of reactive oxidative species production in mitochondria. (cdc.gov)
  • Patients have a homogeneous phenotype characterized by early onset of the disease and clinical presentation of hypotension, psychomotor regression, abnormal eye movements and respiratory failure in combination with brain stem and basal ganglia lesions leading to death. (mpg.de)
  • Abnormal nerve conduction velocities have been recorded in patients with long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency and peripheral neuropathy. (medscape.com)
  • Mitochondrial enzyme studies may reveal abnormal respiratory chain function in skeletal muscle specimens. (medscape.com)
  • This phenomenon was not accompanied by an increase in mitochondrial respiratory activity. (elifesciences.org)
  • At high NADH levels, α-ketoglutarate dehydrogenase (α-KGDH) is a major source of superoxide in skeletal muscle mitochondria with low NNT activity. (uni-wuerzburg.de)
  • Cadmium inhibits the activity of Complexes II and III of respiratory chain and induces ROS production in mitochondria. (cdc.gov)
  • A metabolic specialist needs to confirm or exclude diagnosis of long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency by requesting an acylcarnitine profile and urine organic acid analysis. (medscape.com)