Publications

Found 39 results
Filters: Keyword is Disease Models, Animal  [Clear All Filters]
2017
Hewitt VL & Whitworth AJ (2017) Mechanisms of Parkinson's Disease: Lessons from Drosophila. Curr Top Dev Biol 121, 173-200
2016
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
2015
Yancey DM, Guichard JL, Ahmed MI, Zhou L, Murphy MP, Johnson MS, Benavides GA, Collawn J, Darley-Usmar V & Dell'Italia LJ (2015) Cardiomyocyte mitochondrial oxidative stress and cytoskeletal breakdown in the heart with a primary volume overload. Am J Physiol Heart Circ Physiol 308, H651-63
Huang W, Cash N, Wen L, Szatmary P, Mukherjee R, Armstrong J, Chvanov M, Tepikin AV, Murphy MP, Sutton R & Criddle DN (2015) Effects of the mitochondria-targeted antioxidant mitoquinone in murine acute pancreatitis. Mediators Inflamm 2015, 901780
2014
Bottani E, Giordano C, Civiletto G, Di Meo I, Auricchio A, Ciusani E, Marchet S, Lamperti C, d'Amati G, Viscomi C & Zeviani M (2014) AAV-mediated liver-specific MPV17 expression restores mtDNA levels and prevents diet-induced liver failure. Mol Ther 22, 10-7
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
Torres-Torronteras J, Viscomi C, Cabrera-Pérez R, Cámara Y, Di Meo I, Barquinero J, Auricchio A, Pizzorno G, Hirano M, Zeviani M & Martí R (2014) Gene therapy using a liver-targeted AAV vector restores nucleoside and nucleotide homeostasis in a murine model of MNGIE. Mol Ther 22, 901-7
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
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
Pun PBoon Li, Logan A, Darley-Usmar V, Chacko B, Johnson MS, Huang GW, Rogatti S, Prime TA, Methner C, Krieg T, Fearnley IM, Larsen L, Larsen DS, Menger KE, Collins Y, James AM, Kumar GDKishore, Hartley RC, Smith RAJ & Murphy MP (2014) A mitochondria-targeted mass spectrometry probe to detect glyoxals: implications for diabetes. Free Radic Biol Med 67, 437-50
Cerutti R, Pirinen E, Lamperti C, Marchet S, Sauve AA, Li W, Leoni V, Schon EA, Dantzer F, Auwerx J, Viscomi C & Zeviani M (2014) NAD(+)-dependent activation of Sirt1 corrects the phenotype in a mouse model of mitochondrial disease. Cell Metab 19, 1042-9
Miquel E, Cassina A, Martínez-Palma L, Souza JM, Bolatto C, Rodríguez-Bottero S, Logan A, Smith RAJ, Murphy MP, Barbeito L, Radi R & Cassina P (2014) Neuroprotective effects of the mitochondria-targeted antioxidant MitoQ in a model of inherited amyotrophic lateral sclerosis. Free Radic Biol Med 70, 204-13
Wang A, Keita ÅV, Phan V, McKay CM, Schoultz I, Lee J, Murphy MP, Fernando M, Ronaghan N, Balce D, Yates R, Dicay M, Beck PL, MacNaughton WK, Söderholm JD & McKay DM (2014) Targeting mitochondria-derived reactive oxygen species to reduce epithelial barrier dysfunction and colitis. Am J Pathol 184, 2516-27
2013
Indrieri A, Conte I, Chesi G, Romano A, Quartararo J, Tatè R, Ghezzi D, Zeviani M, Goffrini P, Ferrero I, Bovolenta P & Franco B (2013) The impairment of HCCS leads to MLS syndrome by activating a non-canonical cell death pathway in the brain and eyes. EMBO Mol Med 5, 280-93
Dashdorj A, Jyothi KR, Lim S, Jo A, Nguyen MNam, Ha J, Yoon K-S, Kim HJong, Park J-H, Murphy MP & Kim SSoo (2013) Mitochondria-targeted antioxidant MitoQ ameliorates experimental mouse colitis by suppressing NLRP3 inflammasome-mediated inflammatory cytokines. BMC Med 11, 178
Davies AL, Desai RA, Bloomfield PS, McIntosh PR, Chapple KJ, Linington C, Fairless R, Diem R, Kasti M, Murphy MP & Smith KJ (2013) Neurological deficits caused by tissue hypoxia in neuroinflammatory disease. Ann Neurol 74, 815-25
Zhang L, Karsten P, Hamm S, Pogson JH, A Muller-Rischart K, Exner N, Haass C, Whitworth AJ, Winklhofer KF, Schulz JB & Voigt A (2013) TRAP1 rescues PINK1 loss-of-function phenotypes. Hum Mol Genet 22, 2829-41
2012
Di Meo I, Auricchio A, Lamperti C, Burlina A, Viscomi C & Zeviani M (2012) Effective AAV-mediated gene therapy in a mouse model of ethylmalonic encephalopathy. EMBO Mol Med 4, 1008-14
von Montfort C, Matias N, Fernandez A, Fucho R, de la Rosa LConde, Martinez-Chantar MLuz, Mato JM, Machida K, Tsukamoto H, Murphy MP, Mansouri A, Kaplowitz N, Garcia-Ruiz C & Fernandez-Checa JC (2012) Mitochondrial GSH determines the toxic or therapeutic potential of superoxide scavenging in steatohepatitis. J Hepatol 57, 852-9
Sanchez-Martinez A, Calleja M, Peralta S, Matsushima Y, Hernandez-Sierra R, Whitworth AJ, Kaguni LS & Garesse R (2012) Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects. PLoS One 7, e43954
Giordano C, Viscomi C, Orlandi M, Papoff P, Spalice A, Burlina A, Di Meo I, Tiranti V, Leuzzi V, d'Amati G & Zeviani M (2012) Morphologic evidence of diffuse vascular damage in human and in the experimental model of ethylmalonic encephalopathy. J Inherit Metab Dis 35, 451-8
Pung YFen, Rocic P, Murphy MP, Smith RAJ, Hafemeister J, Ohanyan V, Guarini G, Yin L & Chilian WM (2012) Resolution of mitochondrial oxidative stress rescues coronary collateral growth in Zucker obese fatty rats. Arterioscler Thromb Vasc Biol 32, 325-34
2011
Whitworth AJ (2011) Drosophila models of Parkinson's disease. Adv Genet 73, 1-50
Vlachantoni D, Bramall AN, Murphy MP, Taylor RW, Shu X, Tulloch B, Van Veen T, Turnbull DM, McInnes RR & Wright AF (2011) Evidence of severe mitochondrial oxidative stress and a protective effect of low oxygen in mouse models of inherited photoreceptor degeneration. Hum Mol Genet 20, 322-35
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

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