VISCOMI, CARLO FIORE
 Distribuzione geografica
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Continente #
NA - Nord America 3.940
EU - Europa 442
AS - Asia 389
AF - Africa 12
OC - Oceania 6
SA - Sud America 4
Totale 4.793
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Nazione #
US - Stati Uniti d'America 3.936
CN - Cina 309
IT - Italia 168
DE - Germania 74
SE - Svezia 64
GB - Regno Unito 57
FI - Finlandia 36
VN - Vietnam 26
IE - Irlanda 15
IN - India 13
MA - Marocco 12
ID - Indonesia 11
HK - Hong Kong 9
UA - Ucraina 9
NL - Olanda 8
SG - Singapore 8
BD - Bangladesh 7
AU - Australia 4
CA - Canada 4
BE - Belgio 3
BR - Brasile 3
JO - Giordania 3
CH - Svizzera 2
ES - Italia 2
FR - Francia 2
NZ - Nuova Zelanda 2
IR - Iran 1
JP - Giappone 1
LI - Liechtenstein 1
NO - Norvegia 1
PE - Perù 1
PK - Pakistan 1
Totale 4.793
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Città #
Fairfield 895
Woodbridge 425
Ashburn 367
Seattle 331
Houston 311
Cambridge 294
Wilmington 245
Chandler 225
Ann Arbor 120
San Diego 102
Beijing 95
Padova 77
Medford 73
Princeton 73
Roxbury 56
Des Moines 52
New York 31
Helsinki 30
Shenyang 30
Dong Ket 26
Jinan 25
London 25
Tianjin 22
Dublin 15
Nanjing 15
Hebei 13
Aprilia 12
Zhengzhou 11
Changsha 10
Guangzhou 8
Haikou 8
Fuzhou 7
Raipur 7
Cagliari 6
Nanchang 6
Ningbo 6
Ogden 6
Palangkaraya 6
Taizhou 6
Acton 5
Kilburn 5
Mandi 5
Montgomery 5
Pegirian 5
Rome 5
Taiyuan 5
Tangier 5
Vicenza 5
Farnborough 4
Hangzhou 4
Hong Kong 4
Kowloon 4
Kunming 4
Lappeenranta 4
Milan 4
Norwalk 4
Amman 3
Brussels 3
Cologne 3
Hefei 3
Kharkiv 3
Lanzhou 3
Montesarchio 3
Nijmegen 3
Sydney 3
São Paulo 3
Washington 3
Arzignano 2
Boardman 2
Castegnero 2
Chiswick 2
Curtarolo 2
Downingtown 2
Hackney 2
Hounslow 2
Jiaxing 2
Ksar el Kbir 2
Lancenigo-Villorba 2
Los Angeles 2
Madrid 2
Newcastle Upon Tyne 2
Opfikon 2
Prescot 2
Quinto di Treviso 2
Rudky 2
San Francisco 2
Schio 2
Shanghai 2
Singapore 2
Slough 2
Stockholm 2
Toronto 2
Venice 2
Amsterdam 1
Auckland 1
Baotou 1
Bhalwal 1
Boydton 1
Brescia 1
Buffalo 1
Totale 4.241
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Nome #
A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4−/− Mice 182
The Opa1-Dependent Mitochondrial Cristae Remodeling Pathway Controls Atrophic, Apoptotic and Ischemic Tissue Damage 161
Transcription Factor EB Controls Metabolic Flexibility during Exercise 129
Opa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models 106
MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion 93
Unravelling the role of Fission Protein 1: a forgotten mitochondrial fission factor with pleiotropic roles. 90
Chronic exposure to sulfide causes accelerated degradation of cytochrome c oxidase in ethylmalonic encephalopathy 88
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
Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice 83
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
Kinetic and ionic properties of the human HCN2 pacemaker channel 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
C Terminus-mediated Control of Voltage and cAMP Gating of Hyperpolarization-activated Cyclic Nucleotide-gated Channels 79
Effective AAV-mediated gene therapy in a mouse model of ethylmalonic encephalopathy 75
In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis 74
The homeostatic dynamics of feeding behaviour identify novel mechanisms of anorectic agents 73
Toward a therapy for Mitochondrial disease 71
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
Morphologic evidence of diffuse vascular damage in human and in the experimental model of ethylmalonic encephalopathy 70
A novel heteroplasmic tRNASer(UCN) mtDNA point mutation associated with progressive external ophthalmoplegia and hearing loss 69
Down-regulation of the mitochondrial aspartate-glutamate carrier isoform 1 AGC1 inhibits proliferation and N-acetylaspartate synthesis in Neuro2A cells 68
Niche stiffness underlies the ageing of central nervous system progenitor cells 68
TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III 68
APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS 67
Lactic acidosis in a newborn with adrenal calcifications 66
Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy 66
Cryo-em structures of complex i from mouse heart mitochondria in two biochemically defined states 65
Hypoxic and hypercapnic challenges unveil respiratory vulnerability of Surf1 knockout mice, an animal model of Leigh syndrome 64
Paroxysmal non-kinesigenic dyskinesia is caused by mutations of the MR-1 mitochondrial targeting sequence 64
Control of mitochondrial superoxide production by reverse electron transport at complex I 64
SURF1 knockout cloned pigs: Early onset of a severe lethal phenotype 63
Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration 63
Effects of dronedarone on acetylcholine-activated current in rabbit SAN cells 61
Pharmacological inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle 61
Heteromeric HCN1-HCN4 channels: A comparison with native pacemaker channels from the rabbit sinoatrial node 60
Bioenergetic consequences from xenotopic expression of a tunicate AOX in mouse mitochondria: Switch from RET and ROS to FET 60
Physical and Functional Cross Talk Between Endo-Sarcoplasmic Reticulum and Mitochondria in Skeletal Muscle 59
The short N-terminus is required for functional expression of the virus-encoded miniature K+ channel Kcv 59
Strategies for fighting mitochondrial diseases 59
Defective endoplasmic reticulum-mitochondria contacts and bioenergetics in SEPN1-related myopathy 58
Complex IV-deficient Surf1-/- mice initiate mitochondrial stress responses 57
Evolution meets disease: Penetrance and functional epistasis of mitochondrial tRNA mutations 56
Integrated allosteric model of voltage gating of HCN channels 56
Respiratory chain signalling is essential for adaptive remodelling following cardiac ischaemia 55
Data on cytochrome c oxidase assembly in mice and human fibroblasts or tissues induced by SURF1 defect 53
Interaction of the pacemaker channel HCN1 with filamin A 52
MtDNA-maintenance defects: syndromes and genes 52
Towards a therapy for mitochondrial disease: An update 52
Proteome adaptations in Ethe1-deficient mice indicate a role in lipid catabolism and cytoskeleton organization via post-translational protein modifications 51
Quantitative proteomics suggests metabolic reprogramming during ETHE1 deficiency 51
Opa1 Overexpression Protects from Early-Onset Mpv17−/−-Related Mouse Kidney Disease 51
Erratum: Control of mitochondrial superoxide production by reverse electron transport at complex I. (Journal of Biological Chemistry 2018 293 (9869–9879) DOI: 10.1074/jbc.RA118.003647) 49
Breathe: Your Mitochondria Will Do the Rest… If They Are Healthy! 49
Gene therapy using a liver-targeted AAV vector restores nucleoside and nucleotide homeostasis in a murine model of MNGIE 48
Deep bradycardia and heart block caused by inducible cardiac-specific knockout of the pacemaker channel gene Hcn4 47
Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects 45
FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders 45
Lifelong reduction in complex IV induces tissue-specific metabolic effects but does not reduce lifespan or healthspan in mice 43
Mitochondria in health and disease 43
Effects of ketosis in mitochondrial myopathy: potential benefits of a mitotoxic diet 42
Emerging concepts in the therapy of mitochondrial disease 42
Decreased in vitro mitochondrial function is associated with enhanced brain metabolism, blood flow, and memory in Surf1-deficient mice 41
DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit depletion 40
Metabolic effects of bezafibrate in mitochondrial disease 35
Long-Term Sustained Effect of Liver-Targeted Adeno-Associated Virus Gene Therapy for Mitochondrial Neurogastrointestinal Encephalomyopathy 33
Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer’s disease-like pathology in human cerebral organoids 33
Structural basis for a complex I mutation that blocks pathological ROS production 30
Mitochondrial DNA heteroplasmy is modulated during oocyte development propagating mutation transmission 25
CG7630 is the Drosophila melanogaster homolog of the cytochrome c oxidase subunit COX7B 21
Measurement of mitochondrial respiratory chain enzymatic activities in Drosophila melanogaster samples 15
MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion 14
TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III 12
APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS 12
Bioenergetic consequences from xenotopic expression of a tunicate AOX in mouse mitochondria: Switch from RET and ROS to FET 11
Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration 10
Double administration of self-complementary AAV9(NDUFS4) prevents Leigh disease in Ndufs4(-/-) mice 8
Tissue specific differences in the assembly of mitochondrial complex I is revealed by a novel ENU mutation in ECSIT 8
Mitochondrial Cytochrome c Oxidase Defects Alter Cellular Homeostasis of Transition Metals 8
Redox Signaling and Stress in Inherited Myopathies 6
PPAR-gamma agonist pioglitazone recovers mitochondrial quality control in fibroblasts from PITRM1-deficient patients 6
Measuring the Mitochondrial Ubiquinone (Q) Pool Redox State in Isolated Respiring Mitochondria 4
Gene therapy for primary mitochondrial diseases: experimental advances and clinical challenges 4
Mitochondrial Neurodegeneration 3
Drosophila Mpv17 forms an ion channel and regulates energy metabolism 3
Opportunities for mitochondrial disease gene therapy 3
Rapid fractionation of mitochondria from mouse liver and heart reveals in vivo metabolite compartmentation 3
SURF1 knockout cloned pigs: Early onset of a severe lethal phenotype 3
Molecular Research on Mitochondrial Dysfunction 2
Xenotopic expression of alternative oxidase (AOX) to study mechanisms of mitochondrial disease 2
Zebrafish polg2 knock-out recapitulates human POLG-disorders; implications for drug treatment 1
Mitochondrial DNA Sequencing and Heteroplasmy Quantification by Next Generation Sequencing 1
Role of pitrm1 in mitochondrial dysfunction and neurodegeneration 1
Totale 4.928
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Categoria #
all - tutte 21.013
article - articoli 20.416
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 386
Totale 41.815
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2018/201921 0 0 0 0 0 0 0 0 0 0 10 11
2019/20201.308 6 0 0 4 4 3 198 255 308 283 186 61
2020/20211.316 123 90 181 120 16 25 36 150 185 154 109 127
2021/20221.174 39 126 174 114 32 69 71 134 42 31 111 231
2022/2023601 121 50 28 52 69 66 22 48 71 2 50 22
2023/2024451 33 69 55 49 24 59 43 79 21 15 4 0
Totale 4.928