Found 217 results
Filters: Keyword is Mitochondrial Proteins  [Clear All Filters]
Stettner GM, Viscomi C, Zeviani M, Wilichowski E & Dutschmann M (2011) Hypoxic and hypercapnic challenges unveil respiratory vulnerability of Surf1 knockout mice, an animal model of Leigh syndrome. Mitochondrion 11, 413-20
Viscomi C, Bottani E, Civiletto G, Cerutti R, Moggio M, Fagiolari G, Schon EA, Lamperti C & Zeviani M (2011) In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis. Cell Metab 14, 80-90
Ghezzi D, Arzuffi P, Zordan M, Da Re C, Lamperti C, Benna C, D'Adamo P, Diodato D, Costa R, Mariotti C, Uziel G, Smiderle C & Zeviani M (2011) Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies. Nat Genet 43, 259-63
Hyvärinen AK, Pohjoismäki JLO, Holt IJ & Jacobs HT (2011) Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication. Mol Biol Rep 38, 1321-8
Rorbach J, Nicholls TJJ & Minczuk M (2011) PDE12 removes mitochondrial RNA poly(A) tails and controls translation in human mitochondria. Nucleic Acids Res 39, 7750-63
Deas E, Plun-Favreau H, Gandhi S, Desmond H, Kjaer S, H Y Loh S, Renton AEM, Harvey RJ, Whitworth AJ, L Martins M, Abramov AY & Wood NW (2011) PINK1 cleavage at position A103 by the mitochondrial protease PARL. Hum Mol Genet 20, 867-79
Divakaruni AS & Brand MD (2011) The regulation and physiology of mitochondrial proton leak. Physiology (Bethesda) 26, 192-205
Uusimaa J, Jungbluth H, Fratter C, Crisponi G, Feng L, Zeviani M, Hughes I, Treacy EP, Birks J, Brown GK, Sewry CA, McDermott M, Muntoni F & Poulton J (2011) Reversible infantile respiratory chain deficiency is a unique, genetically heterogenous mitochondrial disease. J Med Genet 48, 660-668
Narendra DP & Youle RJ (2011) Targeting mitochondrial dysfunction: role for PINK1 and Parkin in mitochondrial quality control. Antioxid Redox Signal 14, 1929-38
Minczuk M, He J, Duch AM, Ettema TJ, Chlebowski A, Dzionek K, Nijtmans LGJ, Huynen MA & Holt IJ (2011) TEFM (c17orf42) is necessary for transcription of human mtDNA. Nucleic Acids Res 39, 4284-99
Affourtit C, Jastroch M & Brand MD (2011) Uncoupling protein-2 attenuates glucose-stimulated insulin secretion in INS-1E insulinoma cells by lowering mitochondrial reactive oxygen species. Free Radic Biol Med 50, 609-16
Viscomi C, Burlina AB, Dweikat I, Savoiardo M, Lamperti C, Hildebrandt T, Tiranti V & Zeviani M (2010) Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy. Nat Med 16, 869-71
Azzu V & Brand MD (2010) Degradation of an intramitochondrial protein by the cytosolic proteasome. J Cell Sci 123, 578-85
Ziviani E & Whitworth AJ (2010) How could Parkin-mediated ubiquitination of mitofusin promote mitophagy?. Autophagy 6, 660-2
Chouchani ET, Hurd TR, Nadtochiy SM, Brookes PS, Fearnley IM, Lilley KS, Smith RAJ & Murphy MP (2010) Identification of S-nitrosated mitochondrial proteins by S-nitrosothiol difference in gel electrophoresis (SNO-DIGE): implications for the regulation of mitochondrial function by reversible S-nitrosation. Biochem J 430, 49-59
Requejo R, Chouchani ET, Hurd TR, Menger KE, Hampton MB & Murphy MP (2010) Measuring mitochondrial protein thiol redox state. Methods Enzymol 474, 123-47
Kunji ERS & Crichton PG (2010) Mitochondrial carriers function as monomers. Biochim Biophys Acta 1797, 817-31
Manczak M, Mao P, Calkins MJ, Cornea A, Reddy AP, Murphy MP, Szeto HH, Park B & P Reddy H (2010) Mitochondria-targeted antioxidants protect against amyloid-beta toxicity in Alzheimer's disease neurons. J Alzheimers Dis 20 Suppl 2, S609-31
Kieper N, Holmstrom KM, Ciceri D, Fiesel FC, Wolburg H, Ziviani E, Whitworth AJ, L Martins M, Kahle PJ & Krüger R (2010) Modulation of mitochondrial function and morphology by interaction of Omi/HtrA2 with the mitochondrial fusion factor OPA1. Exp Cell Res 316, 1213-24
Crichton PG, Albury MS, Affourtit C & Moore AL (2010) Mutagenesis of the Sauromatum guttatum alternative oxidase reveals features important for oxygen binding and catalysis. Biochim Biophys Acta 1797, 732-7
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
Azzu V, Mookerjee SA & Brand MD (2010) Rapid turnover of mitochondrial uncoupling protein 3. Biochem J 426, 13-7
Azzu V, Jastroch M, Divakaruni AS & Brand MD (2010) The regulation and turnover of mitochondrial uncoupling proteins. Biochim Biophys Acta 1797, 785-91
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
Toime LJ & Brand MD (2010) Uncoupling protein-3 lowers reactive oxygen species production in isolated mitochondria. Free Radic Biol Med 49, 606-11