Publications

Found 217 results
Filters: Keyword is Mitochondrial Proteins  [Clear All Filters]
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
Johnson MA, Vidoni S, Durigon R, Pearce SF, Rorbach J, He J, Brea-Calvo G, Minczuk M, Reyes A, Holt IJ & Spinazzola A (2014) Amino acid starvation has opposite effects on mitochondrial and cytosolic protein synthesis. PLoS One 9, e93597
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
Suomi F, Menger KE, Monteuuis G, Naumann U, Kursu VASamuli, Shvetsova A & Kastaniotis AJ (2014) Expression and evolution of the non-canonically translated yeast mitochondrial acetyl-CoA carboxylase Hfa1p. PLoS One 9, e114738
Da-Rè C, von Stockum S, Biscontin A, Millino C, Cisotto P, Zordan MA, Zeviani M, Bernardi P, De Pittà C & Costa R (2014) Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencing. J Biol Chem 289, 29235-46
Saez-Atienzar S, Bonet-Ponce L, Blesa JR, Romero FJ, Murphy MP, Jordan J & Galindo MF (2014) The LRRK2 inhibitor GSK2578215A induces protective autophagy in SH-SY5Y cells: involvement of Drp-1-mediated mitochondrial fission and mitochondrial-derived ROS signaling. Cell Death Dis 5, e1368
Sazanov LA (2014) The mechanism of coupling between electron transfer and proton translocation in respiratory complex I. J Bioenerg Biomembr 46, 247-53
Methner C, Chouchani ET, Buonincontri G, Pell VR, Sawiak SJ, Murphy MP & Krieg T (2014) Mitochondria selective S-nitrosation by mitochondria-targeted S-nitrosothiol protects against post-infarct heart failure in mouse hearts. Eur J Heart Fail 16, 712-7
Bingol B, Tea JS, Phu L, Reichelt M, Bakalarski CE, Song Q, Foreman O, Kirkpatrick DS & Sheng M (2014) The mitochondrial deubiquitinase USP30 opposes parkin-mediated mitophagy. Nature 510, 370-5
Sood A, Jeyaraju DVijey, Prudent J, Caron A, Lemieux P, McBride HMay, Laplante M, Tóth K & Pellegrini L (2014) A Mitofusin-2-dependent inactivating cleavage of Opa1 links changes in mitochondria cristae and ER contacts in the postprandial liver. Proc Natl Acad Sci U S A 111, 16017-22
Rosa IDalla, Durigon R, Pearce SF, Rorbach J, Hirst EMA, Vidoni S, Reyes A, Brea-Calvo G, Minczuk M, Woellhaf MW, Herrmann JM, Huynen MA, Holt IJ & Spinazzola A (2014) MPV17L2 is required for ribosome assembly in mitochondria. Nucleic Acids Res 42, 8500-15
Rorbach J, Boesch P, Gammage PA, Nicholls TJJ, Pearce SF, Patel D, Hauser A, Perocchi F & Minczuk M (2014) MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome. Mol Biol Cell 25, 2542-55
Melchionda L, Haack TB, Hardy S, Abbink TEM, Fernandez-Vizarra E, Lamantea E, Marchet S, Morandi L, Moggio M, Carrozzo R, Torraco A, Diodato D, Strom TM, Meitinger T, Tekturk P, Yapici Z, Al-Murshedi F, Stevens R, Rodenburg RJ, Lamperti C, Ardissone A, Moroni I, Uziel G, Prokisch H, Taylor RW, Bertini E, van der Knaap MS, Ghezzi D & Zeviani M (2014) Mutations in APOPT1, encoding a mitochondrial protein, cause cavitating leukoencephalopathy with cytochrome c oxidase deficiency. Am J Hum Genet 95, 315-25
Bason JV, Montgomery MG, Leslie AGW & Walker JE (2014) Pathway of binding of the intrinsically disordered mitochondrial inhibitor protein to F1-ATPase. Proc Natl Acad Sci U S A 111, 11305-10
2013
Kazak L, Reyes A, Duncan AL, Rorbach J, Wood SR, Brea-Calvo G, Gammage PA, Robinson AJ, Minczuk M & Holt IJ (2013) Alternative translation initiation augments the human mitochondrial proteome. Nucleic Acids Res 41, 2354-69
Chouchani ET, Methner C, Nadtochiy SM, Logan A, Pell VR, Ding S, James AM, Cochemé HM, Reinhold J, Lilley KS, Partridge L, Fearnley IM, Robinson AJ, Hartley RC, Smith RAJ, Krieg T, Brookes PS & Murphy MP (2013) Cardioprotection by S-nitrosation of a cysteine switch on mitochondrial complex I. Nat Med 19, 753-9
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
Invernizzi F, Tigano M, Dallabona C, Donnini C, Ferrero I, Cremonte M, Ghezzi D, Lamperti C & Zeviani M (2013) A homozygous mutation in LYRM7/MZM1L associated with early onset encephalopathy, lactic acidosis, and severe reduction of mitochondrial complex III activity. Hum Mutat 34, 1619-22
Deepa SS, Pulliam D, Hill S, Shi Y, Walsh ME, Salmon A, Sloane L, Zhang N, Zeviani M, Viscomi C, Musi N & Van Remmen H (2013) Improved insulin sensitivity associated with reduced mitochondrial complex IV assembly and activity. FASEB J 27, 1371-80
Sánchez E, Lobo T, Fox JL, Zeviani M, Winge DR & Fernandez-Vizarra E (2013) LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells. Biochim Biophys Acta 1827, 285-93
Pearce S, Nezich CLaura & Spinazzola A (2013) Mitochondrial diseases: translation matters. Mol Cell Neurosci 55, 1-12
Palmieri F (2013) The mitochondrial transporter family SLC25: identification, properties and physiopathology. Mol Aspects Med 34, 465-84
Gai X, Ghezzi D, Johnson MA, Biagosch CA, Shamseldin HE, Haack TB, Reyes A, Tsukikawa M, Sheldon CA, Srinivasan S, Gorza M, Kremer LS, Wieland T, Strom TM, Polyak E, Place E, Consugar M, Ostrovsky J, Vidoni S, Robinson AJ, Wong L-J, Sondheimer N, Salih MA, Al-Jishi E, Raab CP, Bean C, Furlan F, Parini R, Lamperti C, Mayr JA, Konstantopoulou V, Huemer M, Pierce EA, Meitinger T, Freisinger P, Sperl W, Prokisch H, Alkuraya FS, Falk MJ & Zeviani M (2013) Mutations in FBXL4, encoding a mitochondrial protein, cause early-onset mitochondrial encephalomyopathy. Am J Hum Genet 93, 482-95
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
Monné M, Palmieri F & Kunji ERS (2013) The substrate specificity of mitochondrial carriers: mutagenesis revisited. Mol Membr Biol 30, 149-59

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