Publications

Found 47 results
Filters: Keyword is Mice, Knockout  [Clear All Filters]
2019
Kim Y-R, Baek J-I, Kim SHwan, Kim M-A, Lee B, Ryu N, Kim K-H, Choi D-G, Kim H-M, Murphy MP, Macpherson G, Choo Y-S, Bok J, Lee K-Y, Park J-W & Kim U-K (2019) Therapeutic potential of the mitochondria-targeted antioxidant MitoQ in mitochondrial-ROS induced sensorineural hearing loss caused by Idh2 deficiency. Redox Biol 20, 544-555
2016
Vrbacký M, Kovalčíková J, Chawengsaksophak K, Beck IM, Mráček T, Nůsková H, Sedmera D, Papoušek F, Kolář F, Sobol M, Hozák P, Sedlacek R & Houštěk J (2016) Knockout of Tmem70 alters biogenesis of ATP synthase and leads to embryonal lethality in mice. Hum Mol Genet 25, 4674-4685
2015
Bee L, Nasca A, Zanolini A, Cendron F, D'Adamo P, Costa R, Lamperti C, Celotti L, Ghezzi D & Zeviani M (2015) A nonsense mutation of human XRCC4 is associated with adult-onset progressive encephalocardiomyopathy. EMBO Mol Med 7, 918-29
Civiletto G, Varanita T, Cerutti R, Gorletta T, Barbaro S, Marchet S, Lamperti C, Viscomi C, Scorrano L & Zeviani M (2015) Opa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models. Cell Metab 21, 845-54
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
Pulliam DA, Deepa SS, Liu Y, Hill S, Lin A-L, Bhattacharya A, Shi Y, Sloane L, Viscomi C, Zeviani M & Van Remmen H (2014) Complex IV-deficient Surf1(-/-) mice initiate mitochondrial stress responses. Biochem J 462, 359-71
Page MM, Sinclair A, Robb EL, Stuart JA, Withers DJ & Selman C (2014) Fibroblasts derived from long-lived insulin receptor substrate 1 null mice are not resistant to multiple forms of stress. Aging Cell 13, 962-4
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
Pirinen E, Cantó C, Jo YSuk, Morato L, Zhang H, Menzies KJ, Williams EG, Mouchiroud L, Moullan N, Hagberg C, Li W, Timmers S, Imhof R, Verbeek J, Pujol A, van Loon B, Viscomi C, Zeviani M, Schrauwen P, Sauve AA, Schoonjans K & Auwerx J (2014) Pharmacological Inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle. Cell Metab 19, 1034-41
Ryu D, Jo YSuk, Sasso GLo, Stein S, Zhang H, Perino A, Lee JUee, Zeviani M, Romand R, Hottiger MO, Schoonjans K & Auwerx J (2014) A SIRT7-dependent acetylation switch of GABPβ1 controls mitochondrial function. Cell Metab 20, 856-869
2013
Lin A-L, Pulliam DA, Deepa SS, Halloran JJ, Hussong SA, Burbank RR, Bresnen A, Liu Y, Podlutskaya N, Soundararajan A, Muir E, Duong TQ, Bokov AF, Viscomi C, Zeviani M, Richardson AG, Van Remmen H, Fox PT & Galvan V (2013) Decreased in vitro mitochondrial function is associated with enhanced brain metabolism, blood flow, and memory in Surf1-deficient mice. J Cereb Blood Flow Metab 33, 1605-11
Yu E, Calvert PA, Mercer JR, Harrison J, Baker L, Figg NL, Kumar S, Wang JC, Hurst LA, Obaid DR, Logan A, West NEJ, Clarke MCH, Vidal-Puig A, Murphy MP & Bennett MR (2013) Mitochondrial DNA damage can promote atherosclerosis independently of reactive oxygen species through effects on smooth muscle cells and monocytes and correlates with higher-risk plaques in humans. Circulation 128, 702-12
Porteous CM, Menon DK, Aigbirhio FI, Smith RAJ & Murphy MP (2013) P-glycoprotein (Mdr1a/1b) and breast cancer resistance protein (Bcrp) decrease the uptake of hydrophobic alkyl triphenylphosphonium cations by the brain. Biochim Biophys Acta 1830, 3458-65
García-Gómez S, Reyes A, Martínez-Jiménez MI, E Chocrón S, Mourón S, Terrados G, Powell C, Salido E, Méndez J, Holt IJ & Blanco L (2013) PrimPol, an archaic primase/polymerase operating in human cells. Mol Cell 52, 541-53
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
Hildebrandt TM, Di Meo I, Zeviani M, Viscomi C & Braun H-P (2013) Proteome adaptations in Ethe1-deficient mice indicate a role in lipid catabolism and cytoskeleton organization via post-translational protein modifications. Biosci Rep 33
2012
Divakaruni AS, Humphrey DM & Brand MD (2012) Fatty acids change the conformation of uncoupling protein 1 (UCP1). J Biol Chem 287, 36845-53
Ashrafian H, Czibik G, Bellahcene M, Aksentijević D, Smith AC, Mitchell SJ, Dodd MS, Kirwan J, Byrne JJ, Ludwig C, Isackson H, Yavari A, Støttrup NB, Contractor H, Cahill TJ, Sahgal N, Ball DR, Birkler RID, Hargreaves I, Tennant DA, Land J, Lygate CA, Johannsen M, Kharbanda RK, Neubauer S, Redwood C, de Cabo R, Ahmet I, Talan M, Günther UL, Robinson AJ, Viant MR, Pollard PJ, Tyler DJ & Watkins H (2012) Fumarate is cardioprotective via activation of the Nrf2 antioxidant pathway. Cell Metab 15, 361-71
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
Mercer JR, Yu E, Figg N, Cheng K-K, Prime TA, Griffin JL, Masoodi M, Vidal-Puig A, Murphy MP & Bennett MR (2012) The mitochondria-targeted antioxidant MitoQ decreases features of the metabolic syndrome in ATM+/-/ApoE-/- mice. Free Radic Biol Med 52, 841-9
2011
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
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
2010
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

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