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Ivano Di Meo
Ivano Di Meo
Fondazione IRCCS Istituto Neurologico Carlo Besta
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Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy
V Tiranti, C Viscomi, T Hildebrandt, I Di Meo, R Mineri, C Tiveron, ...
Nature medicine 15 (2), 200-205, 2009
4222009
Transcription factor EB controls metabolic flexibility during exercise
G Mansueto, A Armani, C Viscomi, L D’Orsi, R De Cegli, EV Polishchuk, ...
Cell metabolism 25 (1), 182-196, 2017
2952017
Chronic exposure to sulfide causes accelerated degradation of cytochrome c oxidase in ethylmalonic encephalopathy
I Di Meo, G Fagiolari, A Prelle, C Viscomi, M Zeviani, V Tiranti
Antioxidants & redox signaling 15 (2), 353-362, 2011
952011
Effective AAV‐mediated gene therapy in a mouse model of ethylmalonic encephalopathy
I Di Meo, A Auricchio, C Lamperti, A Burlina, C Viscomi, M Zeviani
EMBO molecular medicine 4 (9), 1008-1014, 2012
902012
SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder
V Del Dotto, F Ullah, I Di Meo, P Magini, M Gusic, A Maresca, L Caporali, ...
The Journal of clinical investigation 130 (1), 108-125, 2020
772020
Dissection of metabolic reprogramming in polycystic kidney disease reveals coordinated rewiring of bioenergetic pathways
C Podrini, I Rowe, R Pagliarini, ASH Costa, M Chiaravalli, I Di Meo, H Kim, ...
Communications biology 1 (1), 194, 2018
752018
Classification and molecular pathogenesis of NBIA syndromes
I Di Meo, V Tiranti
european journal of paediatric neurology 22 (2), 272-284, 2018
742018
Liver transplant in ethylmalonic encephalopathy: a new treatment for an otherwise fatal disease
C Dionisi-Vici, D Diodato, G Torre, S Picca, R Pariante, ...
Brain 139 (4), 1045-1051, 2016
742016
Gene therapy using a liver-targeted AAV vector restores nucleoside and nucleotide homeostasis in a murine model of MNGIE
J Torres-Torronteras, C Viscomi, R Cabrera-Pérez, Y Cámara, I Di Meo, ...
Molecular therapy 22 (5), 901-907, 2014
742014
Identification of new mutations in the ETHE1 gene in a cohort of 14 patients presenting with ethylmalonic encephalopathy
R Mineri, M Rimoldi, AB Burlina, S Koskull, C Perletti, B Heese, ...
Journal of medical genetics 45 (7), 473-478, 2008
712008
AAV9-based gene therapy partially ameliorates the clinical phenotype of a mouse model of Leigh syndrome
I Di Meo, S Marchet, C Lamperti, M Zeviani, C Viscomi
Gene therapy 24 (10), 661-667, 2017
702017
Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway
M Ziosi, I Di Meo, G Kleiner, XH Gao, E Barca, MJ Sanchez‐Quintero, ...
EMBO molecular medicine 9 (1), 96-111, 2017
682017
AAV-mediated liver-specific MPV17 expression restores mtDNA levels and prevents diet-induced liver failure
E Bottani, C Giordano, G Civiletto, I Di Meo, A Auricchio, E Ciusani, ...
Molecular Therapy 22 (1), 10-17, 2014
582014
Mitochondrial diseases caused by toxic compound accumulation: from etiopathology to therapeutic approaches
I Di Meo, C Lamperti, V Tiranti
EMBO molecular medicine 7 (10), 1257-1266, 2015
522015
Ethylmalonic encephalopathy: application of improved biochemical and molecular diagnostic approaches
A Drousiotou, I DiMeo, R Mineri, T Georgiou, G Stylianidou, V Tiranti
Clinical genetics 79 (4), 385-390, 2011
462011
Morphologic evidence of diffuse vascular damage in human and in the experimental model of ethylmalonic encephalopathy
C Giordano, C Viscomi, M Orlandi, P Papoff, A Spalice, A Burlina, I Di Meo, ...
Journal of inherited metabolic disease 35, 451-458, 2012
442012
Inborn errors of coenzyme A metabolism and neurodegeneration
I Di Meo, M Carecchio, V Tiranti
Journal of Inherited Metabolic Disease 42 (1), 49-56, 2019
412019
Proteome adaptations in Ethe1-deficient mice indicate a role in lipid catabolism and cytoskeleton organization via post-translational protein modifications
TM Hildebrandt, I Di Meo, M Zeviani, C Viscomi, HP Braun
Bioscience Reports 33 (4), e00052, 2013
352013
Response to medical and a novel dietary treatment in newborn screen identified patients with ethylmalonic encephalopathy
M Boyer, M Sowa, I Di Meo, S Eftekharian, MR Steenari, V Tiranti, ...
Molecular Genetics and Metabolism 124 (1), 57-63, 2018
332018
SURF1 knockout cloned pigs: Early onset of a severe lethal phenotype
C Quadalti, D Brunetti, I Lagutina, R Duchi, A Perota, G Lazzari, R Cerutti, ...
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1864 (6 …, 2018
322018
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