Found 90 results
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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
Letts JA, Fiedorczuk K & Sazanov LA (2016) The architecture of respiratory supercomplexes. Nature 537, 644-648
McCorvie TJ, Kopec J, Pey AL, Fitzpatrick F, Patel D, Chalk R, Shrestha L & Yue WW (2016) Molecular basis of classic galactosemia from the structure of human galactose 1-phosphate uridylyltransferase. Hum Mol Genet 25, 2234-2244
Lee J, Ding S, Walpole TB, Holding AN, Montgomery MG, Fearnley IM & Walker JE (2015) Organization of Subunits in the Membrane Domain of the Bovine F-ATPase Revealed by Covalent Cross-linking. J Biol Chem 290, 13308-20
Zhou A, Rohou A, Schep DG, Bason JV, Montgomery MG, Walker JE, Grigorieff N & Rubinstein JL (2015) Structure and conformational states of the bovine mitochondrial ATP synthase by cryo-EM. Elife 4, e10180
Shirakihara Y, Shiratori A, Tanikawa H, Nakasako M, Yoshida M & Suzuki T (2015) Structure of a thermophilic F1-ATPase inhibited by an ε-subunit: deeper insight into the ε-inhibition mechanism. FEBS J 282, 2895-913
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
Xu Y, Tao Y, Cheung LS, Fan C, Chen L-Q, Xu S, Perry K, Frommer WB & Feng L (2014) Structures of bacterial homologues of SWEET transporters in two distinct conformations. Nature 515, 448-52
Ruprecht JJ, Hellawell AM, Harding M, Crichton PG, McCoy AJ & Kunji ERS (2014) Structures of yeast mitochondrial ADP/ATP carriers support a domain-based alternating-access transport mechanism. Proc Natl Acad Sci U S A 111, E426-34
Alavian KN, Beutner G, Lazrove E, Sacchetti S, Park H-A, Licznerski P, Li H, Nabili P, Hockensmith K, Graham M, Porter GA & Jonas EA (2014) An uncoupling channel within the c-subunit ring of the F1FO ATP synthase is the mitochondrial permeability transition pore. Proc Natl Acad Sci U S A 111, 10580-5
Runswick MJ, Bason JV, Montgomery MG, Robinson GC, Fearnley IM & Walker JE (2013) The affinity purification and characterization of ATP synthase complexes from mitochondria. Open Biol 3, 120160
Walker JE (2013) The ATP synthase: the understood, the uncertain and the unknown. Biochem Soc Trans 41, 1-16
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
Palmieri F (2013) The mitochondrial transporter family SLC25: identification, properties and physiopathology. Mol Aspects Med 34, 465-84
Baruffini E, Dallabona C, Invernizzi F, Yarham JW, Melchionda L, Blakely EL, Lamantea E, Donnini C, Santra S, Vijayaraghavan S, Roper HP, Burlina A, Kopajtich R, Walther A, Strom TM, Haack TB, Prokisch H, Taylor RW, Ferrero I, Zeviani M & Ghezzi D (2013) MTO1 mutations are associated with hypertrophic cardiomyopathy and lactic acidosis and cause respiratory chain deficiency in humans and yeast. Hum Mutat 34, 1501-9
Narendra DP, Wang C, Youle RJ & Walker JE (2013) PINK1 rendered temperature sensitive by disease-associated and engineered mutations. Hum Mol Genet 22, 2572-89
Robinson GC, Bason JV, Montgomery MG, Fearnley IM, Mueller DM, Leslie AGW & Walker JE (2013) The structure of F₁-ATPase from Saccharomyces cerevisiae inhibited by its regulatory protein IF₁. Open Biol 3, 120164
Baker LA, Watt IN, Runswick MJ, Walker JE & Rubinstein JL (2012) Arrangement of subunits in intact mammalian mitochondrial ATP synthase determined by cryo-EM. Proc Natl Acad Sci U S A 109, 11675-80
Rinaldi C, Grunseich C, Sevrioukova IF, Schindler A, Horkayne-Szakaly I, Lamperti C, Landouré G, Kennerson ML, Burnett BG, Bönnemann C, Biesecker LG, Ghezzi D, Zeviani M & Fischbeck KH (2012) Cowchock syndrome is associated with a mutation in apoptosis-inducing factor. Am J Hum Genet 91, 1095-102
Divakaruni AS, Humphrey DM & Brand MD (2012) Fatty acids change the conformation of uncoupling protein 1 (UCP1). J Biol Chem 287, 36845-53
Bason JV, Runswick MJ, Fearnley IM & Walker JE (2011) Binding of the inhibitor protein IF(1) to bovine F(1)-ATPase. J Mol Biol 406, 443-53
Cingolani G & Duncan TM (2011) Structure of the ATP synthase catalytic complex (F(1)) from Escherichia coli in an autoinhibited conformation. Nat Struct Mol Biol 18, 701-7
Mizutani K, Yamamoto M, Suzuki K, Yamato I, Kakinuma Y, Shirouzu M, Walker JE, Yokoyama S, Iwata S & Murata T (2011) Structure of the rotor ring modified with N,N'-dicyclohexylcarbodiimide of the Na+-transporting vacuolar ATPase. Proc Natl Acad Sci U S A 108, 13474-9
Kunji ERS & Crichton PG (2010) Mitochondrial carriers function as monomers. Biochim Biophys Acta 1797, 817-31
Requejo R, Chouchani ET, James AM, Prime TA, Lilley KS, Fearnley IM & Murphy MP (2010) Quantification and identification of mitochondrial proteins containing vicinal dithiols. Arch Biochem Biophys 504, 228-35