ZEVIANI, MASSIMO
 Distribuzione geografica
Continente #
NA - Nord America 19.335
EU - Europa 1.446
AS - Asia 763
SA - Sud America 19
AF - Africa 15
OC - Oceania 13
Continente sconosciuto - Info sul continente non disponibili 2
Totale 21.593
Nazione #
US - Stati Uniti d'America 19.317
CN - Cina 556
IT - Italia 315
FI - Finlandia 258
SE - Svezia 232
DE - Germania 203
GB - Regno Unito 202
VN - Vietnam 118
UA - Ucraina 87
IE - Irlanda 52
NL - Olanda 40
IN - India 26
HK - Hong Kong 19
FR - Francia 16
CA - Canada 15
BR - Brasile 14
MA - Marocco 13
ID - Indonesia 11
ES - Italia 9
SG - Singapore 9
BD - Bangladesh 7
NZ - Nuova Zelanda 7
AU - Australia 6
SI - Slovenia 6
PS - Palestinian Territory 5
NO - Norvegia 4
RU - Federazione Russa 4
TR - Turchia 4
BE - Belgio 3
JO - Giordania 3
PT - Portogallo 3
RO - Romania 3
BG - Bulgaria 2
CH - Svizzera 2
CO - Colombia 2
CR - Costa Rica 2
EU - Europa 2
HU - Ungheria 2
IR - Iran 2
PE - Perù 2
AT - Austria 1
AZ - Azerbaigian 1
DZ - Algeria 1
EC - Ecuador 1
EG - Egitto 1
HR - Croazia 1
IL - Israele 1
JP - Giappone 1
MX - Messico 1
RS - Serbia 1
Totale 21.593
Città #
Fairfield 4.193
Woodbridge 1.952
Ashburn 1.771
Houston 1.641
Seattle 1.599
Cambridge 1.427
Chandler 1.205
Wilmington 1.200
Ann Arbor 706
San Diego 498
Princeton 471
Medford 423
Des Moines 312
Roxbury 224
Beijing 217
Helsinki 214
Jacksonville 179
Dong Ket 118
Padova 106
London 92
Dublin 52
New York 50
Ogden 50
Nanjing 44
Shenyang 39
Boardman 38
Kharkiv 34
Jinan 26
Kilburn 24
Norwalk 23
Tianjin 22
Nijmegen 20
Guangzhou 15
Washington 15
Aprilia 14
Hebei 14
Borås 13
Hong Kong 12
Rockville 12
Rome 12
São Paulo 12
Chiswick 11
Milan 11
Nanchang 11
Zhengzhou 11
Haikou 10
Redwood City 10
Berlin 9
Changsha 9
Jiaxing 9
Pune 9
Ningbo 8
Prescot 8
Selvazzano Dentro 8
Amsterdam 7
Chicago 7
Falls Church 7
Gavardo 7
Hounslow 7
Raipur 7
Taizhou 7
Acton 6
Bologna 6
Ferrara 6
Lappeenranta 6
Madrid 6
Miami Beach 6
Munich 6
New Bedfont 6
Palangkaraya 6
Sommacampagna 6
Brescia 5
Islington 5
Kunming 5
Los Angeles 5
Montgomery 5
Pegirian 5
Ramallah 5
Southwark 5
Stockholm 5
Tangier 5
Voghera 5
Dalsjoefors 4
Frankfurt am Main 4
Hangzhou 4
Istanbul 4
Kowloon 4
Pinehaven 4
Sydney 4
Taiyuan 4
Toronto 4
Venice 4
Amman 3
Auckland 3
Calgary 3
Fuzhou 3
Glasgow 3
Hefei 3
High Bridge 3
Mandi 3
Totale 19.441
Nome #
A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4−/− Mice 187
Impaired Mitochondrial ATP Production Downregulates Wnt Signaling via ER Stress Induction 172
The Opa1-Dependent Mitochondrial Cristae Remodeling Pathway Controls Atrophic, Apoptotic and Ischemic Tissue Damage 161
Effects of pirenzepine on plasma insulin, glucagon and pancreatic polypeptide levels in normal man. 136
"Mitochondrial neuropathies": A survey from the large cohort of the Italian Network 136
Transcription Factor EB Controls Metabolic Flexibility during Exercise 129
The m.3243A>G mitochondrial DNA mutation and related phenotypes. A matter of gender? 128
Post-transcriptional silencing and functional characterization of the Drosophila melanogaster homolog of human Surf1 122
Myoclonus in mitochondrial disorders. 115
Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis 115
Cowchock syndrome is associated with a mutation in apoptosis-inducing factor 114
Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice. 113
Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice 113
Fatal infantile cytochrome c oxidase deficiency: Decrease of immunologically detectable enzyme in muscle 113
A nonsense mutation of human XRCC4 is associated with adult-onset progressive encephalocardiomyopathy 112
A single cell complementation class is common to several cases of Cytochrome c Oxidase defective Leigh's syndrome 109
Opa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models 106
Expression of the Ciona intestinalis Alternative Oxidase (AOX) in Drosophila Complements Defects in Mitochondrial Oxidative Phosphorylation 105
Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies. 99
A direct repeat is a hotspot for large-scale deletion of human mitochondrial DNA. 97
MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion 97
Epileptic phenotypes associated with mitochondrial disorders 96
Clinical and biochemical studies on cytochrome oxidase deficiencies. 93
Partial tandem duplication of mtDNA-tRNA Phe impairs mtDNA translation in late-onset mitochondrial myopathy 93
Reduced mitochondrial Ca 2+ transients stimulate autophagy in human fibroblasts carrying the 13514A>G mutation of the ND5 subunit of NADH dehydrogenase 92
Molecular defects in cytochrome oxidase in mitochondrial diseases. 91
Myofibrillar-protein isoforms and sarcoplasmic-reticulum Ca++-transport activity of single human muscle fibres 91
Redefining phenotypes associated with mitochondrial DNA single deletion 91
Cloning and expression of human nebulin cDNAs and assignment of the gene to chromosome 2q31-q32. 90
Phenotypic heterogeneity of the 8344A>G mtDNA "MERRF" mutation. 89
Reduced mitochondrial Ca2+ transients stimulate autophagy in human fibroblasts carrying the 13514A>G mutation of the ND5 subunit of NADH dehydrogenase 89
Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy 89
Variable penetrance of a familial progressive necrotising encephalopathy due to a novel tRNA(Ile) homoplasmic mutation in the mitochondrial genome 89
Neuromuscular syndrome associated with the 3291T→C mutation of mitochondrial DNA: A second case 89
UCP4C mediates uncoupled respiration in larvae of Drosophila melanogaster 88
Chronic exposure to sulfide causes accelerated degradation of cytochrome c oxidase in ethylmalonic encephalopathy 88
PINK1 heterozygous rare variants: Prevalence, significance and phenotypic spectrum 87
Severe early onset ethylmalonic encephalopathy with West syndrome 86
Complete loss-of-function of the heart/muscle-specific adenine nucleotide translocator is associated with mitochondrial myopathy and cardiomyopathy 86
Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy 85
AAV9-based gene therapy partially ameliorates the clinical phenotype of a mouse model of Leigh syndrome 85
SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy 85
Revisiting mitochondrial ocular myopathies: a study from the Italian Network 84
Unravelling the effects of the mutation m.3571insC/MT-ND1 on respiratory complexes structural organization 84
A third locus predisposing to multiple deletions of mtDNA in autosomal dominant progressive external ophthalmoplegia [5] 84
Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice 83
Evidence that nebulin is a protein-ruler in muscle thin filaments 83
Mitochondrial PITRM1 peptidase loss-of-function in childhood cerebellar atrophy 82
Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy 82
AAV-mediated liver-specific MPV17 expression restores mtdna levels and prevents diet-induced liver failure 81
Myopathy and Fatal Cardiopathy due to Cytochrome c Oxidase Deficiency 81
Exploiting pyocyanin to treat mitochondrial disease due to respiratory complex III dysfunction 81
Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo 80
Subunit Va of human and bovine cytochrome c oxidase is highly conserved. 79
Sequence of cDNAs encoding subunit Vb of human and bovine cytochrome c oxidase. 79
Disease-causing SDHAF1 mutations impair transfer of Fe-S clusters to SDHB 79
NUCLEAR-DNA ORIGIN OF CYTOCHROME-C-OXIDASE DEFICIENCY IN LEIGHS SYNDROME - GENETIC-EVIDENCE BASED ON PATIENTS-DERIVED RHO-DEGREES TRANSFORMANTS 77
Compound heterozygous missense and deep intronic variants in NDUFAF6 unraveled by exome sequencing and mRNA analysis 77
Neuronal complex I deficiency occurs throughout the Parkinson’s disease brain, but is not associated with neurodegeneration or mitochondrial DNA damage 77
Defects in maintenance of mitochondrial DNA are associated with intramitochondrial nucleotide imbalances 77
Foxg1 localizes to mitochondria and coordinates cell differentiation and bioenergetics 76
Effective AAV-mediated gene therapy in a mouse model of ethylmalonic encephalopathy 75
Mitochondrial medicine: A metabolic perspective on the pathology of oxidative phosphorylation disorders 75
Mutations in TIMM50 compromise cell survival in OxPhos-dependent metabolic conditions 75
In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis 74
Knockdown of APOPT1/COA8 Causes Cytochrome c Oxidase Deficiency, Neuromuscular Impairment, and Reduced Resistance to Oxidative Stress in Drosophila melanogaster 73
Differential expression of genes specifying two isoforms of subunit VIa of human cytochrome c oxidase. 72
Functional Characterization of drim2, the Drosophila melanogaster Homolog of the Yeast Mitochondrial Deoxynucleotide Transporter 72
Clinical, biochemical and genetic spectrum of 70 patients with ACAD9 deficiency: Is riboflavin supplementation effective? 72
Cavitating Leukoencephalopathy With Posterior Predominance Caused by a Deletion in the APOPT1 Gene in an Indian Boy 72
Expression of the SMN gene, the spinal muscular atrophy determining gene, in the mammalian central nervous system 72
A novel mutation (8342G → A) in the mitochondrial tRNA(Lys) gene associated with progressive external ophthalmoplegia and myoclonus 72
Disorders from perturbations of nuclear-mitochondrial intergenomic cross-talk 72
X-inactivation patterns in female Leber's hereditary optic neuropathy patients do not support a strong X-linked determinant. 70
Localized cerebral energy failure in DNA polymerase gamma-associated encephalopathy syndromes 70
NAD+-dependent activation of Sirt1 corrects the phenotype in a mouse model of mitochondrial disease 70
Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis 70
Fatal infantile mitochondrial myopathy and renal dysfunction caused by cytochrome c oxidase deficiency: Immunological studies in a new patient 70
EFNS task force on molecular diagnosis of neurologic disorders: Guidelines for the molecular diagnosis of inherited neurologic diseases. First of two parts 70
Morphologic evidence of diffuse vascular damage in human and in the experimental model of ethylmalonic encephalopathy 70
A two-nuclease pathway involving RNase H1 is required for primer removal at human mitochondrial OriL 69
Lipomatosis incidence and characteristics in an Italian cohort of mitochondrial patients 69
MRI in leigh syndrome with SURF1 gene mutation [3] 69
Down-regulation of the mitochondrial aspartate-glutamate carrier isoform 1 AGC1 inhibits proliferation and N-acetylaspartate synthesis in Neuro2A cells 68
TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III 68
Mitochondrial encephalomyopathies 67
Human diseases associated with defects in assembly of OXPHOS complexes 67
Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV 67
APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS 67
Mitochondrial complex III Rieske Fe-S protein processing and assembly 66
Lactic acidosis in a newborn with adrenal calcifications 66
Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy 66
MTO1 mutations are associated with hypertrophic cardiomyopathy and lactic acidosis and cause respiratory chain deficiency in humans and yeast 66
Adult-onset Alexander disease, associated with a mutation in an alternative GFAP transcript, may be phenotypically modulated by a non-neutral HDAC6 variant 66
Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencing. 65
Mitochondrial Myopathy due to Complex III Deficiency With Normal Reducible Cytochrome b Concentration 65
Mutations of SURF-1 in Leigh disease associated with cytochrome C oxidase deficiency 65
Dominantly inherited mitochondrial myopathy with multiple deletions of mitochondrial DNA: Clinical, morphologic, and biochemical studies 65
Fulminant Leigh syndrome and sudden unexpected death in a family with the T9176C mutation of the mitochondrial ATPase 6 gene 65
Hypoxic and hypercapnic challenges unveil respiratory vulnerability of Surf1 knockout mice, an animal model of Leigh syndrome 64
Totale 8.673
Categoria #
all - tutte 103.266
article - articoli 101.733
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 317
Totale 205.316


Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2018/2019339 0 0 0 0 0 0 0 0 0 0 187 152
2019/20202.217 130 66 26 135 147 94 260 329 371 339 224 96
2020/20219.472 120 128 152 4.849 617 242 190 426 1.106 249 953 440
2021/20225.245 79 231 718 242 619 259 115 625 643 85 464 1.165
2022/20232.874 793 256 81 285 374 349 23 182 318 23 132 58
2023/20241.289 71 192 176 140 123 172 133 98 97 59 28 0
Totale 22.117