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Bi-allelic Mutations in NDUFA6 Establish Its Role in Early-Onset Isolated Mitochondrial Complex I Deficiency.
Charlotte L Alston, Juliana Heidler, Marris G Dibley, Laura S Kremer, Lucie S Taylor, Carl Fratter, Courtney E French, Ruth IC Glasgow, René G Feichtinger, Isabelle Delon, Alistair T Pagnamenta, Helen Dolling, Hugh Lemonde, Neil Aiton, Alf Bjørnstad, Lisa Henneke, Jutta Gärtner, Holger Thiele, Katerina Tauchmannova, Gerardine Quaghebeur, Josef Houstek, Wolfgang Sperl, F Lucy Raymond, Holger Prokisch, Johannes A Mayr, Robert McFarland, Joanna Poulton, Michael T Ryan, Ilka Wittig, Marco Henneke, Robert W Taylor
May 21, 2019
Isolated complex I deficiency is a common biochemical phenotype observed in pediatric mitochondrial disease and often arises as a consequence of pathogenic variants affecting one of the ∼65 genes encoding the complex I...
Neuronal complex I deficiency occurs throughout the Parkinson's disease brain, but is not associated with neurodegeneration or mitochondrial DNA damage.
Irene H Flønes, Erika Fernandez-Vizarra, Maria Lykouri, Brage Brakedal, Geir Olve Skeie, Hrvoje Miletic, Peer K Lilleng, Guido Alves, Ole-Bjørn Tysnes, Kristoffer Haugarvoll, Christian Dölle, Massimo Zeviani, Charalampos Tzoulis
Mar 22, 2018
Mitochondrial complex I deficiency occurs in the substantia nigra of individuals with Parkinson's disease. It is generally believed that this phenomenon is caused by accumulating mitochondrial DNA damage in neurons and that it...
Complex I Mitochondria Parkinsonism Respiratory chain Substantia Nigra Aged Aged 80 and over Brain DNA Damage DNA Mitochondrial Electron Transport Complex I Female Humans Male Middle Aged Mitochondria Mitochondrial Diseases Nerve Degeneration Neurons Parkinson Disease Prospective Studies protein aggregation Pathological ALPHA-SYNUCLEIN
Suppression of reactive oxygen species generation in heart mitochondria from anoxic turtles
Freshwater turtles (Trachemys scripta) are among the very few vertebrates capable of tolerating severe hypoxia and re-oxygenation without suffering from damage to the heart. As myocardial ischemia and reperfusion causes a burst...
Loss of COX4I1 Leads to Combined Respiratory Chain Deficiency and Impaired Mitochondrial Protein Synthesis.
Kristýna Čunátová, David Pajuelo Reguera, Marek Vrbacký, Erika Fernández-Vizarra, ShuJing Ding, Ian M Fearnley, Massimo Zeviani, Josef Houštěk, Tomáš Mráček, Petr Pecina
Feb 11, 2021
Cox COX4 OXPHOS biogenesis interdependency CI cIV cIV assembly Complex I complexome profiling knock-out Mitochondria mitochondrial protein synthesis Electron Transport Complex IV Glycolysis HEK293 Cells Humans Mitochondrial Diseases Mitochondrial Proteins Oxidative Phosphorylation Oxygen Consumption Protein Biosynthesis Protein Subunits
Control of mitochondrial superoxide production by reverse electron transport at complex I.
Ellen L Robb, Andrew R Hall, Tracy A Prime, Simon Eaton, Marten Szibor, Carlo Viscomi, Andrew M James, Michael P Murphy
Dec 17, 2018
The generation of mitochondrial superoxide (O2̇̄) by reverse electron transport (RET) at complex I causes oxidative damage in pathologies such as ischemia reperfusion injury, but also provides the precursor to H2O2 production in...
RET coenzyme Q Complex I Mitochondria mitochondrial membrane potential reactive oxygen species (ROS) redox signaling Respiration reverse electron transport Superoxide Animals Electron Transport Electron Transport Complex I Female Hydrogen Peroxide Male Mice Mice Inbred C57BL Mitochondria Heart Oxidative Phosphorylation Rats Rats Wistar Signal Transduction Superoxides Ubiquinone
CHCHD4 regulates tumour proliferation and EMT-related phenotypes, through respiratory chain-mediated metabolism
Luke W. Thomas, Cinzia Esposito, Jenna M. Stephen, Ana S. H. Costa, Christian Frezza, Thomas S. Blacker, Gyorgy Szabadkai, Margaret Ashcroft
Jul 15, 2020
Abstract: Background: Mitochondrial oxidative phosphorylation (OXPHOS) via the respiratory chain is required for the maintenance of tumour cell proliferation and regulation of epithelial to mesenchymal transition (EMT)-related...
Post-translational modifications near the quinone binding site of mammalian complex I.
Complex I (NADH:ubiquinone oxidoreductase) in mammalian mitochondria is an L-shaped assembly of 44 protein subunits with one arm buried in the inner membrane of the mitochondrion and the orthogonal arm protruding about 100 Å...
Arginine Modification Bioenergetics/Electron Transfer Complex Complex I Electron Transport System (ETS) Mitochondria Protein Chemical Modification protein hydroxylation Protein Methylation Animals Bacterial Proteins Binding Sites Cattle Electron Transport Complex I Flavin Mononucleotide Fungal Proteins HEK293 Cells Humans Lipid Bilayers Mitochondria Heart NAD Paracoccus denitrificans Pichia Structural Homology Protein Thermus thermophilus Ubiquinone
NDUFAF7 methylates arginine 85 in the NDUFS2 subunit of human complex I.
Complex I (NADH ubiquinone oxidoreductase) in mammalian mitochondria is an L-shaped assembly of 44 subunits. One arm is embedded in the inner membrane with the other protruding ∼100 Å into the matrix of the organelle. The...
assembly Bioenergetics Complex I Dimethylarginine Electron Transport Methyltransferase Mitochondria Protein Methylation Respiratory chain Arginine Cell Line Tumor Humans Methylation Methyltransferases NADH Dehydrogenase Protein Folding Protein Structure Secondary Protein-Arginine N-Methyltransferases
CHCHD4 confers metabolic vulnerabilities to tumour cells through its control of the mitochondrial respiratory chain.
Luke W Thomas, Jenna M Stephen, Cinzia Esposito, Simon Hoer, Robin Antrobus, Afshan Ahmed, Hasan Al-Habib, Margaret Ashcroft
Feb 15, 2019
BACKGROUND: Tumour cells rely on glycolysis and mitochondrial oxidative phosphorylation (OXPHOS) to survive. Thus, mitochondrial OXPHOS has become an increasingly attractive area for therapeutic exploitation in cancer. However...
Respiratory Complex I in Bos taurus and Paracoccus denitrificans Pumps Four Protons across the Membrane for Every NADH Oxidized.
Respiratory complex I couples electron transfer between NADH and ubiquinone to proton translocation across an energy-transducing membrane to support the proton-motive force that drives ATP synthesis. The proton-pumping...
Complex I electron transfer complex Mitochondria mitochondrial respiratory chain complex Oxidative Phosphorylation proton motive force Adenosine Triphosphate Animals Cattle Electron Transport Electron Transport Complex I Mitochondria NAD Oxidation-Reduction Oxidative Phosphorylation Paracoccus denitrificans Proton-Motive Force Protons
NDUFAF5 Hydroxylates NDUFS7 at an Early Stage in the Assembly of Human Complex I.
A conserved arginine residue is critical for stabilizing the N2 FeS cluster in mitochondrial complex I.
Mikhail A Hameedi, Daniel N Grba, Katherine H Richardson, Andrew JY Jones, Wei Song, Maxie M Roessler, John J Wright, Judy Hirst
May 17, 2021
Respiratory complex I (NADH:ubiquinone oxidoreductase), the first enzyme of the electron-transport chain, captures the free energy released by NADH oxidation and ubiquinone reduction to translocate protons across an...
CHCHD4 regulates tumour proliferation and EMT-related phenotypes, through respiratory chain-mediated metabolism.
Luke W Thomas, Cinzia Esposito, Jenna M Stephen, Ana SH Costa, Christian Frezza, Thomas S Blacker, Gyorgy Szabadkai, Margaret Ashcroft
Jun 28, 2019
BACKGROUND: Mitochondrial oxidative phosphorylation (OXPHOS) via the respiratory chain is required for the maintenance of tumour cell proliferation and regulation of epithelial to mesenchymal transition (EMT)-related phenotypes...
Mitochondrial complex I derived ROS regulate stress adaptation in Drosophila melanogaster.
Filippo Scialò, Ashwin Sriram, Rhoda Stefanatos, Ruth V Spriggs, Samantha HY Loh, L Miguel Martins, Alberto Sanz
Feb 26, 2021
Reactive Oxygen Species (ROS) are essential cellular messengers required for cellular homeostasis and regulate the lifespan of several animal species. The main site of ROS production is the mitochondrion, and within it...