Found 39 results
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Steele HE, Harris E, Barresi R, Marsh J, Beattie A, Bourke JP, Straub V & Chinnery PF (2016) Cardiac involvement in hereditary myopathy with early respiratory failure: A cohort study. Neurology 87, 1031-5
Olsen RKJ, Koňaříková E, Giancaspero TA, Mosegaard S, Boczonadi V, Mataković L, Veauville-Merllié A, Terrile C, Schwarzmayr T, Haack TB, Auranen M, Leone P, Galluccio M, Imbard A, Gutierrez-Rios P, Palmfeldt J, Graf E, Vianey-Saban C, Oppenheim M, Schiff M, Pichard S, Rigal O, Pyle A, Chinnery PF, Konstantopoulou V, Möslinger D, Feichtinger RG, Talim B, Topaloglu H, Coskun T, Gucer S, Botta A, Pegoraro E, Malena A, Vergani L, Mazzà D, Zollino M, Ghezzi D, Acquaviva C, Tyni T, Boneh A, Meitinger T, Strom TM, Gregersen N, Mayr JA, Horvath R, Barile M & Prokisch H (2016) Riboflavin-Responsive and -Non-responsive Mutations in FAD Synthase Cause Multiple Acyl-CoA Dehydrogenase and Combined Respiratory-Chain Deficiency. Am J Hum Genet 98, 1130-45
Reyes A, Melchionda L, Nasca A, Carrara F, Lamantea E, Zanolini A, Lamperti C, Fang M, Zhang J, Ronchi D, Bonato S, Fagiolari G, Moggio M, Ghezzi D & Zeviani M (2015) RNASEH1 Mutations Impair mtDNA Replication and Cause Adult-Onset Mitochondrial Encephalomyopathy. Am J Hum Genet 97, 186-93
Bonneau B, Nougarède A, Prudent J, Popgeorgiev N, Peyriéras N, Rimokh R & Gillet G (2014) The Bcl-2 homolog Nrz inhibits binding of IP3 to its receptor to control calcium signaling during zebrafish epiboly. Sci Signal 7, ra14
Carraro M, Giorgio V, Šileikytė J, Sartori G, Forte M, Lippe G, Zoratti M, Szabó I & Bernardi P (2014) Channel formation by yeast F-ATP synthase and the role of dimerization in the mitochondrial permeability transition. J Biol Chem 289, 15980-5
Feillet-Coudray C, Fouret G, Elle REbabe, Rieusset J, Bonafos B, Chabi B, Crouzier D, Zarkovic K, Zarkovic N, Ramos J, Badia E, Murphy MP, Cristol JPaul & Coudray C (2014) The mitochondrial-targeted antioxidant MitoQ ameliorates metabolic syndrome features in obesogenic diet-fed rats better than Apocynin or Allopurinol. Free Radic Res 48, 1232-46
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
Crichton PG, Harding M, Ruprecht JJ, Lee Y & Kunji ERS (2013) Lipid, detergent, and Coomassie Blue G-250 affect the migration of small membrane proteins in blue native gels: mitochondrial carriers migrate as monomers not dimers. J Biol Chem 288, 22163-73
Methner C, Lukowski R, Grube K, Loga F, Smith RAJ, Murphy MP, Hofmann F & Krieg T (2013) Protection through postconditioning or a mitochondria-targeted S-nitrosothiol is unaffected by cardiomyocyte-selective ablation of protein kinase G. Basic Res Cardiol 108, 337
Di Meo I, Fagiolari G, Prelle A, Viscomi C, Zeviani M & Tiranti V (2011) Chronic exposure to sulfide causes accelerated degradation of cytochrome c oxidase in ethylmalonic encephalopathy. Antioxid Redox Signal 15, 353-62
Requejo R, Chouchani ET, Hurd TR, Menger KE, Hampton MB & Murphy MP (2010) Measuring mitochondrial protein thiol redox state. Methods Enzymol 474, 123-47
Frelet-Barrand A, Boutigny S, Kunji ERS & Rolland N (2010) Membrane protein expression in Lactococcus lactis. Methods Mol Biol 601, 67-85
Dallabona C, Marsano RMassimilia, Arzuffi P, Ghezzi D, Mancini P, Zeviani M, Ferrero I & Donnini C (2010) Sym1, the yeast ortholog of the MPV17 human disease protein, is a stress-induced bioenergetic and morphogenetic mitochondrial modulator. Hum Mol Genet 19, 1098-107
Mineri R, Pavelka N, Fernandez-Vizarra E, Ricciardi-Castagnoli P, Zeviani M & Tiranti V (2009) How do human cells react to the absence of mitochondrial DNA?. PLoS One 4, e5713
Crichton PG, Parker N, Vidal-Puig AJ & Brand MD (2009) Not all mitochondrial carrier proteins support permeability transition pore formation: no involvement of uncoupling protein 1. Biosci Rep 30, 187-92
Ghezzi D, Viscomi C, Ferlini A, Gualandi F, Mereghetti P, DeGrandis D & Zeviani M (2009) Paroxysmal non-kinesigenic dyskinesia is caused by mutations of the MR-1 mitochondrial targeting sequence. Hum Mol Genet 18, 1058-64
Ricart KC, Bolisetty S, Johnson MS, Perez J, Agarwal A, Murphy MP & Landar A (2009) The permissive role of mitochondria in the induction of haem oxygenase-1 in endothelial cells. Biochem J 419, 427-36
Whitworth AJ, Lee JR, Ho VM-W, Flick R, Chowdhury R & G McQuibban A (2008) Rhomboid-7 and HtrA2/Omi act in a common pathway with the Parkinson's disease factors Pink1 and Parkin. Dis Model Mech 1, 168-74; discussion 173
Bugiani M, Lamantea E, Invernizzi F, Moroni I, Bizzi A, Zeviani M & Uziel G (2006) Effects of riboflavin in children with complex II deficiency. Brain Dev 28, 576-81
Tiranti V, Briem E, Lamantea E, Mineri R, Papaleo E, De Gioia L, Forlani F, Rinaldo P, Dickson P, Abu-Libdeh B, Cindro-Heberle L, Owaidha M, Jack RM, Christensen E, Burlina A & Zeviani M (2006) ETHE1 mutations are specific to ethylmalonic encephalopathy. J Med Genet 43, 340-6
Helmerhorst EJ, Stan M, Murphy MP, Sherman F & Oppenheim FG (2005) The concomitant expression and availability of conventional and alternative, cyanide-insensitive, respiratory pathways in Candida albicans. Mitochondrion 5, 200-11
Meulener M, Whitworth AJ, Armstrong-Gold CE, Rizzu P, Heutink P, Wes PD, Pallanck LJ & Bonini NM (2005) Drosophila DJ-1 mutants are selectively sensitive to environmental toxins associated with Parkinson's disease. Curr Biol 15, 1572-7
Bustos DM & Velours J (2005) The modification of the conserved GXXXG motif of the membrane-spanning segment of subunit g destabilizes the supramolecular species of yeast ATP synthase. J Biol Chem 280, 29004-10
Chan KWai, Slotboom D-J, Cox S, T Embley M, Fabre O, van der Giezen M, Harding M, Horner DS, Kunji ERS, León-Avila G & Tovar J (2005) A novel ADP/ATP transporter in the mitosome of the microaerophilic human parasite Entamoeba histolytica. Curr Biol 15, 737-42