Publications

Found 118 results
Filters: Keyword is Electron Transport Complex I  [Clear All Filters]
2017
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
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
Zieliński ŁP, Smith AC, Smith AG & Robinson AJ (2016) Metabolic flexibility of mitochondrial respiratory chain disorders predicted by computer modelling. Mitochondrion 31, 45-55
Zhu J, Vinothkumar KR & Hirst J (2016) Structure of mammalian respiratory complex I. Nature 536, 354-8
2015
van Rahden VA, Fernandez-Vizarra E, Alawi M, Brand K, Fellmann F, Horn D, Zeviani M & Kutsche K (2015) Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome. Am J Hum Genet 96, 640-50
Zhu J, King MS, Yu M, Klipcan L, Leslie AGW & Hirst J (2015) Structure of subcomplex Iβ of mammalian respiratory complex I leads to new supernumerary subunit assignments. Proc Natl Acad Sci U S A 112, 12087-92
2014
Vinothkumar KR, Zhu J & Hirst J (2014) Architecture of mammalian respiratory complex I. Nature 515, 80-4
Chouchani ET, Methner C, Buonincontri G, Hu C-H, Logan A, Sawiak SJ, Murphy MP & Krieg T (2014) Complex I deficiency due to selective loss of Ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy. PLoS One 9, e94157
Bridges HR, J Y Jones A, Pollak MN & Hirst J (2014) Effects of metformin and other biguanides on oxidative phosphorylation in mitochondria. Biochem J 462, 475-87
Chouchani ET, Pell VR, Gaude E, Aksentijević D, Sundier SY, Robb EL, Logan A, Nadtochiy SM, Ord ENJ, Smith AC, Eyassu F, Shirley R, Hu C-H, Dare AJ, James AM, Rogatti S, Hartley RC, Eaton S, Costa ASH, Brookes PS, Davidson SM, Duchen MR, Saeb-Parsy K, Shattock MJ, Robinson AJ, Work LM, Frezza C, Krieg T & Murphy MP (2014) Ischaemic accumulation of succinate controls reperfusion injury through mitochondrial ROS. Nature 515, 431-5
Blaza JN, Serreli R, J Y Jones A, Mohammed K & Hirst J (2014) Kinetic evidence against partitioning of the ubiquinone pool and the catalytic relevance of respiratory-chain supercomplexes. Proc Natl Acad Sci U S A 111, 15735-40
Sazanov LA (2014) The mechanism of coupling between electron transfer and proton translocation in respiratory complex I. J Bioenerg Biomembr 46, 247-53
Methner C, Chouchani ET, Buonincontri G, Pell VR, Sawiak SJ, Murphy MP & Krieg T (2014) Mitochondria selective S-nitrosation by mitochondria-targeted S-nitrosothiol protects against post-infarct heart failure in mouse hearts. Eur J Heart Fail 16, 712-7
Diodato D, Melchionda L, Haack TB, Dallabona C, Baruffini E, Donnini C, Granata T, Ragona F, Balestri P, Margollicci M, Lamantea E, Nasca A, Powell CA, Minczuk MA, Strom TM, Meitinger T, Prokisch H, Lamperti C, Zeviani M & Ghezzi D (2014) VARS2 and TARS2 mutations in patients with mitochondrial encephalomyopathies. Hum Mutat 35, 983-9
2013
Andrews B, Carroll J, Ding S, Fearnley IM & Walker JE (2013) Assembly factors for the membrane arm of human complex I. Proc Natl Acad Sci U S A 110, 18934-9
Chouchani ET, Methner C, Nadtochiy SM, Logan A, Pell VR, Ding S, James AM, Cochemé HM, Reinhold J, Lilley KS, Partridge L, Fearnley IM, Robinson AJ, Hartley RC, Smith RAJ, Krieg T, Brookes PS & Murphy MP (2013) Cardioprotection by S-nitrosation of a cysteine switch on mitochondrial complex I. Nat Med 19, 753-9
Baradaran R, Berrisford JM, Minhas GS & Sazanov LA (2013) Crystal structure of the entire respiratory complex I. Nature 494, 443-8
Birrell JA, Morina K, Bridges HR, Friedrich T & Hirst J (2013) Investigating the function of [2Fe-2S] cluster N1a, the off-pathway cluster in complex I, by manipulating its reduction potential. Biochem J 456, 139-46
Birrell JA & Hirst J (2013) Investigation of NADH binding, hydride transfer, and NAD(+) dissociation during NADH oxidation by mitochondrial complex I using modified nicotinamide nucleotides. Biochemistry 52, 4048-55
Sazanov LA, Baradaran R, Efremov RG, Berrisford JM & Minhas G (2013) A long road towards the structure of respiratory complex I, a giant molecular proton pump. Biochem Soc Trans 41, 1265-71
Hirst J (2013) Mitochondrial complex I. Annu Rev Biochem 82, 551-75
Carroll J, Ding S, Fearnley IM & Walker JE (2013) Post-translational modifications near the quinone binding site of mammalian complex I. J Biol Chem 288, 24799-808

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