Publications

Found 621 results
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2019
Małecki JM, Willemen HLDM, Pinto R, Y Y Ho A, Moen A, Kjønstad IF, Burgering BMT, Zwartkruis F, Eijkelkamp N & Falnes PØ (2019) Lysine methylation by the mitochondrial methyltransferase FAM173B optimizes the function of mitochondrial ATP synthase. J Biol Chem 294, 1128-1141
Galván-Peña S, Carroll RG, Newman C, Hinchy EC, Palsson-McDermott E, Robinson EK, Covarrubias S, Nadin A, James AM, Haneklaus M, Carpenter S, Kelly VP, Murphy MP, Modis LK & O'Neill LA (2019) Malonylation of GAPDH is an inflammatory signal in macrophages. Nat Commun 10, 338
Escribano-Lopez I, Bañuls C, Diaz-Morales N, Iannantuoni F, Rovira-Llopis S, Gomis R, Rocha M, Hernandez-Mijares A, Murphy MP & Víctor VM (2019) The Mitochondria-Targeted Antioxidant MitoQ Modulates Mitochondrial Function and Endoplasmic Reticulum Stress in Pancreatic β Cells Exposed to Hyperglycaemia. Cell Physiol Biochem 52, 186-197
Kohlhauer M, Pell VR, Burger N, Spiroski AM, Gruszczyk A, Mulvey JF, Mottahedin A, Costa ASH, Frezza C, Ghaleh B, Murphy MP, Tissier R & Krieg T (2019) Protection against cardiac ischemia-reperfusion injury by hypothermia and by inhibition of succinate accumulation and oxidation is additive. Basic Res Cardiol 114, 18
Posse V, Al-Behadili A, Uhler JP, Clausen AR, Reyes A, Zeviani M, Falkenberg M & Gustafsson CM (2019) RNase H1 directs origin-specific initiation of DNA replication in human mitochondria. PLoS Genet 15, e1007781
Kim Y-R, Baek J-I, Kim SHwan, Kim M-A, Lee B, Ryu N, Kim K-H, Choi D-G, Kim H-M, Murphy MP, Macpherson G, Choo Y-S, Bok J, Lee K-Y, Park J-W & Kim U-K (2019) Therapeutic potential of the mitochondria-targeted antioxidant MitoQ in mitochondrial-ROS induced sensorineural hearing loss caused by Idh2 deficiency. Redox Biol 20, 544-555
2018
Lovero D, Giordano L, Marsano RMassimilia, Sanchez-Martinez A, Boukhatmi H, Drechsler M, Oliva M, Whitworth AJ, Porcelli D & Caggese C (2018) Characterization of Drosophila ATPsynC mutants as a new model of mitochondrial ATP synthase disorders. PLoS One 13, e0201811
Agip A-NA, Blaza JN, Bridges HR, Viscomi C, Rawson S, Muench SP & Hirst J (2018) Cryo-EM structures of complex I from mouse heart mitochondria in two biochemically defined states. Nat Struct Mol Biol 25, 548-556
Hofherr A, Seger C, Fitzpatrick F, Busch T, Michel E, Luan J, Osterried L, Linden F, Kramer-Zucker A, Wakimoto B, Schütze C, Wiedemann N, Artati A, Adamski J, Walz G, Kunji ERS, Montell C, Watnick T & Köttgen M (2018) The mitochondrial transporter SLC25A25 links ciliary TRPP2 signaling and cellular metabolism. PLoS Biol 16, e2005651
Leslie M (2018) 'Old' genome editors might treat mitochondrial diseases. Science 361, 1302
Majd H, King MS, Palmer SM, Smith AC, Elbourne LDh, Paulsen IT, Sharples D, Henderson PJf & Kunji ERs (2018) Screening of candidate substrates and coupling ions of transporters by thermostability shift assays. Elife 7
Rocha MC & Springett R (2018) Spectral components of detergent-solubilized bovine cytochrome oxidase. Biochim Biophys Acta Bioenerg 1859, 555-566
Garone C & Viscomi C (2018) Towards a therapy for mitochondrial disease: an update. Biochem Soc Trans 46, 1247-1261
2017
Feichtinger RG, Oláhová M, Kishita Y, Garone C, Kremer LS, Yagi M, Uchiumi T, Jourdain AA, Thompson K, D'Souza AR, Kopajtich R, Alston CL, Koch J, Sperl W, Mastantuono E, Strom TM, Wortmann SB, Meitinger T, Pierre G, Chinnery PF, Chrzanowska-Lightowlers ZM, Lightowlers RN, DiMauro S, Calvo SE, Mootha VK, Moggio M, Sciacco M, Comi GP, Ronchi D, Murayama K, Ohtake A, Rebelo-Guiomar P, Kohda M, Kang D, Mayr JA, Taylor RW, Okazaki Y, Minczuk MA & Prokisch H (2017) Biallelic C1QBP Mutations Cause Severe Neonatal-, Childhood-, or Later-Onset Cardiomyopathy Associated with Combined Respiratory-Chain Deficiencies. Am J Hum Genet 101, 525-538
Manole A, Jaunmuktane Z, Hargreaves I, Ludtmann MHR, Salpietro V, Bello OD, Pope S, Pandraud A, Horga A, Scalco RS, Li A, Ashokkumar B, Lourenço CM, Heales S, Horvath R, Chinnery PF, Toro C, Singleton AB, Jacques TS, Abramov AY, Muntoni F, Hanna MG, Reilly MM, Revesz T, Kullmann DM, Jepson JEC & Houlden H (2017) Clinical, pathological and functional characterization of riboflavin-responsive neuropathy. Brain 140, 2820-2837
Mühleip AW, Dewar CE, Schnaufer A, Kühlbrandt W & Davies KM (2017) In situ structure of trypanosomal ATP synthase dimer reveals a unique arrangement of catalytic subunits. Proc Natl Acad Sci U S A 114, 992-997
Nesci S, Trombetti F, Ventrella V, Pirini M & Pagliarani A (2017) Kinetic properties of the mitochondrial F1FO-ATPase activity elicited by Ca(2+) in replacement of Mg(2). Biochimie 140, 73-81
Hewitt VL & Whitworth AJ (2017) Mechanisms of Parkinson's Disease: Lessons from Drosophila. Curr Top Dev Biol 121, 173-200
Crichton PG, Lee Y & Kunji ERS (2017) The molecular features of uncoupling protein 1 support a conventional mitochondrial carrier-like mechanism. Biochimie 134, 35-50
Bénit P, Pelhaître A, Saunier E, Bortoli S, Coulibaly A, Rak M, Schiff M, Kroemer G, Zeviani M & Rustin P (2017) Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency. EBioMedicine 17, 75-87
Tsuchiya Y, Peak-Chew SYeu, Newell C, Miller-Aidoo S, Mangal S, Zhyvoloup A, Bakovic J, Malanchuk O, Pereira GC, Kotiadis V, Szabadkai G, Duchen MR, Campbell M, Cuenca SRodriguez, Vidal-Puig A, James AM, Murphy MP, Filonenko V, Skehel M & Gout I (2017) Protein CoAlation: a redox-regulated protein modification by coenzyme A in mammalian cells. Biochem J 474, 2489-2508
J Y Jones A, Blaza JN, Varghese F & Hirst J (2017) Respiratory Complex I in Bos taurus and Paracoccus denitrificans Pumps Four Protons across the Membrane for Every NADH Oxidized. J Biol Chem 292, 4987-4995
2016
Letts JA, Fiedorczuk K & Sazanov LA (2016) The architecture of respiratory supercomplexes. Nature 537, 644-648
Fiedorczuk K, Letts JA, Degliesposti G, Kaszuba K, Skehel M & Sazanov LA (2016) Atomic structure of the entire mammalian mitochondrial complex I. Nature 538, 406-410
Lehtonen JM, Forsström S, Bottani E, Viscomi C, Baris OR, Isoniemi H, Höckerstedt K, Österlund P, Hurme M, Jylhävä J, Leppä S, Markkula R, Heliö T, Mombelli G, Uusimaa J, Laaksonen R, Laaksovirta H, Auranen M, Zeviani M, Smeitink J, Wiesner RJ, Nakada K, Isohanni P & Suomalainen A (2016) FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders. Neurology 87, 2290-2299

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