IRIS Institutional Research Information System - AIR Archivio Istituzionale della Ricerca

CORTI, STEFANIA PAOLA
 Distribuzione geografica
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Continente #
EU - Europa 26.683
NA - Nord America 20.590
AS - Asia 13.149
SA - Sud America 1.352
OC - Oceania 291
AF - Africa 178
Continente sconosciuto - Info sul continente non disponibili 39
Totale 62.282
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Nazione #
US - Stati Uniti d'America 19.405
GB - Regno Unito 7.766
CN - Cina 5.666
IT - Italia 5.032
DE - Germania 4.169
SE - Svezia 2.644
SG - Singapore 2.558
RU - Federazione Russa 1.152
FR - Francia 1.006
IN - India 940
CA - Canada 935
BR - Brasile 894
JP - Giappone 848
IE - Irlanda 767
TR - Turchia 708
FI - Finlandia 644
KR - Corea 641
UA - Ucraina 615
ES - Italia 506
NL - Olanda 467
HK - Hong Kong 429
ID - Indonesia 354
EU - Europa 332
DK - Danimarca 290
BE - Belgio 270
AU - Australia 263
VN - Vietnam 205
CO - Colombia 204
CH - Svizzera 203
GR - Grecia 202
PL - Polonia 201
TW - Taiwan 195
MX - Messico 168
AT - Austria 141
PT - Portogallo 130
RO - Romania 107
AR - Argentina 101
CZ - Repubblica Ceca 77
NO - Norvegia 72
IL - Israele 68
IR - Iran 64
CL - Cile 59
SA - Arabia Saudita 55
EG - Egitto 44
HU - Ungheria 44
MY - Malesia 44
TH - Thailandia 41
UZ - Uzbekistan 36
PH - Filippine 35
HR - Croazia 34
PE - Perù 30
PK - Pakistan 30
EC - Ecuador 28
NZ - Nuova Zelanda 28
DZ - Algeria 27
MA - Marocco 26
BG - Bulgaria 25
CY - Cipro 21
PR - Porto Rico 20
SC - Seychelles 20
AE - Emirati Arabi Uniti 19
KZ - Kazakistan 18
AZ - Azerbaigian 17
LK - Sri Lanka 17
TN - Tunisia 17
IQ - Iraq 15
JO - Giordania 14
DO - Repubblica Dominicana 13
KG - Kirghizistan 13
RS - Serbia 13
SI - Slovenia 13
SK - Slovacchia (Repubblica Slovacca) 13
AM - Armenia 12
LB - Libano 12
NP - Nepal 12
PA - Panama 12
ZA - Sudafrica 12
BO - Bolivia 11
EE - Estonia 11
UY - Uruguay 11
AP - ???statistics.table.value.countryCode.AP??? 10
CR - Costa Rica 10
GT - Guatemala 10
LV - Lettonia 10
QA - Qatar 10
BD - Bangladesh 9
GE - Georgia 9
PY - Paraguay 8
CI - Costa d'Avorio 7
IS - Islanda 7
LT - Lituania 7
AL - Albania 6
BY - Bielorussia 6
LU - Lussemburgo 6
MK - Macedonia 6
MO - Macao, regione amministrativa speciale della Cina 6
VE - Venezuela 6
MD - Moldavia 5
PS - Palestinian Territory 5
A2 - ???statistics.table.value.countryCode.A2??? 4
Totale 62.508
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Città #
Southend 6.379
Chandler 2.087
Singapore 1.784
Milan 1.603
Seattle 1.087
Ashburn 1.029
Fairfield 1.021
Beijing 1.009
Wilmington 943
Princeton 797
Dublin 732
Hanover 726
Ann Arbor 702
Houston 643
Council Bluffs 629
Frankfurt am Main 603
Redwood City 597
Woodbridge 595
Toronto 529
Nanjing 426
Jacksonville 401
Cambridge 370
Shanghai 358
Helsinki 316
Redmond 303
Bengaluru 299
Dearborn 294
Des Moines 287
Mountain View 280
Santa Clara 272
Boardman 256
Jakarta 250
Hong Kong 248
New York 243
Phoenix 226
Jinan 212
Rome 205
Tokyo 197
Ottawa 188
Guangzhou 186
Sakarya 177
Bogotá 169
Andover 163
Brussels 156
Somerville 154
Los Angeles 153
Shenyang 152
Grafing 150
Changsha 147
Serra 142
Hangzhou 139
Istanbul 134
Berlin 125
London 124
Warsaw 124
Fuzhou 116
Nanchang 115
Athens 112
Dong Ket 99
São Paulo 99
Hebei 98
Paris 98
Taipei 98
Wuhan 98
Kiez 95
San Diego 95
Bitonto 94
Munich 90
Tianjin 88
Medford 86
Zhengzhou 83
Vienna 81
Madrid 80
Chicago 75
Ningbo 70
Nuremberg 68
Seoul 67
Chengdu 65
Silver Spring 65
Turin 63
Eitensheim 61
Atlanta 59
Boston 59
Zurich 55
Bologna 52
Nürnberg 52
Sydney 52
Jiaxing 50
Mumbai 50
Amsterdam 48
Melbourne 48
Philadelphia 48
Washington 47
Naples 46
Norwalk 46
Quanzhou 45
Ankara 44
Genoa 44
Shenzhen 44
Buenos Aires 43
Totale 34.712
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Nome #
Silence superoxide dismutase 1 (SOD1): a promising therapeutic target for amyotrophic lateral sclerosis (ALS) 3.637
Time Is Motor Neuron: Therapeutic Window and Its Correlation with Pathogenetic Mechanisms in Spinal Muscular Atrophy 1.757
The Italian limb girdle muscular dystrophy registry : relative frequency, clinical features, and differential diagnosis 1.359
MicroRNA Metabolism and Dysregulation in Amyotrophic Lateral Sclerosis 1.121
Therapeutic Strategies Under Development Targeting Inflammatory Mechanisms in Amyotrophic Lateral Sclerosis 713
Development of Therapeutics for C9ORF72 ALS/FTD-related disorders 687
R-Loops in Motor Neuron Diseases 635
Insights into disease mechanisms and potential therapeutics for C9orf72-related amyotrophic lateral sclerosis/frontotemporal dementia 574
Current understanding of and emerging treatment options for spinal muscular atrophy with respiratory distress type 1 (SMARD1) 543
A de novo C19orf12 heterozygous mutation in a patient with MPAN 466
Decline of ALS-FRS and Norris scale scores in a group of patients affected by ALS and treated with lithium 431
Noncoding RNAs in Duchenne and Becker muscular dystrophies: role in pathogenesis and future prognostic and therapeutic perspectives 431
Dysregulation of Muscle-Specific MicroRNAs as Common Pathogenic Feature Associated with Muscle Atrophy in ALS, SMA and SBMA: Evidence from Animal Models and Human Patients 391
Loss of the nucleoporin Aladin in central nervous system and fibroblasts of Allgrove Syndrome 385
Clinical studies in stem cells transplantation for stroke: a review 347
Advances, Challenges, and Perspectives in Translational Stem Cell Therapy for Amyotrophic Lateral Sclerosis 342
Minimally invasive transplantation of iPSC-derived ALDHhiSSCloVLA4+ neural stem cells effectively improves the phenotype of an amyotrophic lateral sclerosis model 341
Neurofascin (NFASC) gene mutation causes autosomal recessive ataxia with demyelinating neuropathy 341
RAS mutations vontribute to evolution of chronic myelomonocytic leukemia to the proliferative variant 337
La canalopatia del cloro : diagnosi clinica differenziale 336
Preconditioning and Cellular Engineering to Increase the Survival of Transplanted Neural Stem Cells for Motor Neuron Disease Therapy 335
SLC25A46 mutations in patients with Parkinson's Disease and optic atrophy 298
Autophagy in motor neuron disease: Key pathogenetic mechanisms and therapeutic targets 295
Sumoylation regulates the assembly and activity of the SMN complex 286
Key role of SMN/SYNCRIP and RNA-Motif 7 in spinal muscular atrophy: RNA-Seq and motif analysis of human motor neurons 282
miR-129-5p: A key factor and therapeutic target in amyotrophic lateral sclerosis 277
Herpes Simplex virus type 2 myeloradiculitis with a pure motor presentationin a liver transplant recipient 273
Purkinje cell COX deficiency and mtDNA depletion in an animal model of spinocerebellar ataxia type 1 268
Molecular Approaches for the Treatment of Pompe Disease 267
Beta-lactam antibiotic offers neuroprotection in a spinal muscular atrophy model by multiple mechanisms 266
Colocalization of ribonuclear inclusions with muscle blind like-proteins in a family with myotonic dystrophy type 2 associated with a short CCTG expansion 260
Spinal muscular atrophy phenotype is ameliorated in human motor neurons by SMN increase via different novel RNA therapeutic approaches 260
Estimating the impact of COVID-19 pandemic on services provided by Italian Neuromuscular Centers: an Italian Association of Myology survey of the acute phase 259
The mitochondrial disulfide relay system protein GFER is mutated in autosomal recessive myopathy with congenital cataract and COX deficiency. 256
Gene therapy rescues disease phenotype in a spinal muscular atrophy with respiratory distress type 1 (SMARD1) mouse model 248
Mitochondrial Dysregulation and Impaired Autophagy in iPSC-Derived Dopaminergic Neurons of Multiple System Atrophy 248
Regional spreading pattern is associated with clinical phenotype in amyotrophic lateral sclerosis 245
Can Intestinal Pseudo-Obstruction Drive Recurrent Stroke-Like Episodes in Late-Onset MELAS Syndrome? A Case Report and Review of the Literature 232
Morpholino oligomers ameliorates pathological hallmarks in C9orf72 cellular lines and mice 230
Analysis of hnRNPA1, A2/B1, and A3 genes in patients with amyotrophic lateral sclerosis 228
Back to the origins : human brain organoids to investigate neurodegeneration 223
IPSC-derived neural stem cells act via kinase inhibition to exert neuroprotective effects in spinal muscular atrophy with respiratory distress type 1 221
Mutations in TMEM230 are rare in autosomal dominant Parkinson's disease 221
Clinical Phenotype of Pediatric and Adult Patients With Spinal Muscular Atrophy With Four SMN2 Copies: Are They Really All Stable? 218
Clinical and molecular characterization of a cohort of patients with novel nucleotide alterations of the Dystrophin gene detected by direct sequencing 216
Transplanted ALDH(hi)SSC(lo) neural stem cells generate motor neurons and delay disease progression of nmd mice, an animal model of SMARD1 213
Direct reprogramming of adult somatic cells into other lineages: past evidence and future perspectives 212
ISPD mutations account for a small proportion of Italian Limb Girdle Muscular Dystrophy cases 205
Central nervous system involvement in common variable immunodeficiency: A case of acute unilateral optic neuritis in a 26 -year-old Italian Patient 205
Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology 204
Advances in spinal muscular atrophy therapeutics 203
Ophthalmoplegia due to Miller Fisher syndrome in a patient with myasthenia gravis 203
Motor neuron derivation from human embryonic and induced pluripotent stem cells: experimental approaches and clinical perspectives 202
Expanding the genotypic and phenotypic spectrum of Beta-propeller-associated neurodegeneration (BPAN) 202
A cortically blind patient with preserved visual imagery 201
A novel missense mutation of IGHMBP2 gene in an Italian infant with spinal muscular atrophy with respiratory distress type 1 200
Human induced pluripotent stem cell models for the study and treatment of Duchenne and Becker muscular dystrophies 200
Screening of the PFN1 gene in sporadic amyotrophic lateral sclerosis and in frontotemporal dementia 191
Differential neuronal vulnerability identifies IGF-2 as a protective factor in ALS 191
Morpholino-mediated SOD1 reduction ameliorates an amyotrophic lateral sclerosis disease phenotype 191
Neural stem cell transplantation for neurodegenerative diseases 189
A novel homozygous VPS11 variant may cause generalized dystonia 189
Caratterizzazione molecolare dei geni CLCN1, SCN4A, KCNJ2, CACNA1S in pazienti con canalopatie muscolari 187
A case report of late-onset cerebellar ataxia associated with a rare p.R342W TGM6 (SCA35) mutation 187
ALDH positive neural stem cells generate motor neurons and promote functional recovery in NMD mice, an animal model of SMARD1 186
New molecular findings in congenital myopathies due to selenoprotein N gene mutations 186
Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications? 186
Systematic elucidation of neuron-astrocyte interaction in models of amyotrophic lateral sclerosis using multi-modal integrated bioinformatics workflow 184
Stop codons, duplicazioni e delezioni: caratterizzazione genetica e follow-up clinico in una coorte di 201 pazienti affetti da distrofia Muscolare di Duchenne 183
iPSC-Based Models to Unravel Key Pathogenetic Processes Underlying Motor Neuron Disease Development 183
Nusinersen treatment and cerebrospinal fluid neurofilaments : An explorative study on Spinal Muscular Atrophy type 3 patients 183
MicroRNA expression analysis identifies a subset of downregulated miRNAs in ALS motor neuron progenitors 182
A splicing site OPA1 mutation associated with autosomal dominant optic atrophy in an Italian family 181
Limb girdle muscular dystrophy due to LAMA2 gene mutations: new mutations expand the clinical spectrum of a still challenging diagnosis 181
MicroRNAs as regulators of cell death mechanisms in amyotrophic lateral sclerosis 179
The mitochondrial disulfide relay system protein GFER is mutated in sutosomal-tecessive myopathy with vataract and vombined respiratory-chain deficiency 178
In vitro models of multiple system atrophy from primary cells to induced pluripotent stem cells 177
Motor neurons with differential vulnerability to degeneration show distinct protein signatures in health and ALS 176
Glial cells involvement in spinal muscular atrophy: Could SMA be a neuroinflammatory disease? 176
Mitochondrial DNA G8363A mutation in the tRNA Lys gene: clinical, biochemical and pathological study 175
A novel MAPT mutation associated to late-onset spinal muscular atrophy without dementia in a large Italian family 175
STIM1 R304W in mice causes subgingival hair growth and an increased fraction of trabecular bone 175
Extracellular vesicles and amyotrophic lateral sclerosis : from misfolded protein vehicles to promising clinical biomarkers 171
Subclinical Leber's hereditary optic neuropathy with pediatric acute spinal cord onset: More than meets the eye 170
A novel CCM1 mutation associated with multiple cerebral and vertebral cavernous malformations 168
Bilateral Cavernous Carotid Aneurysms: Atypical Presentation of a Rare Cause of Mass Effect. A Case Report and a Review of the Literature 168
Management of patients with neuromuscular disorders at the time of the SARS-CoV-2 pandemic 168
Glucose-free/high-protein diet improves hepatomegaly and exercise intolerance in glycogen storage disease type III mice 168
Inclusion body myopathy and frontotemporal dementia caused by a novel VCP mutation 167
Skin-derived stem cells transplanted into resorable guides provide functional nerve regeneration after sciatic nerve resection 167
Novel Q23R SOD1 mutation associated with muscle mitochondrial dysfunction 166
POLG1 mutations and stroke like episodes : a distinct clinical entity rather than an atypical MELAS syndrome 166
Diagnostic and Prognostic Role of Blood and Cerebrospinal Fluid and Blood Neurofilaments in Amyotrophic Lateral Sclerosis: A Review of the Literature 166
ALS genetic modifiers that increase survival of SOD1 mice and are suitable for therapeutic development 164
Amyotrophic lateral sclerosis linked to a novel SOD1 mutation with muscle mitochondrial dysfunction 163
Myotonia congenita : novel mutations in CLCN1 gene and functional characterizations in Italian patients 163
5'azacytidine enhances exogenous gene expression in muscle 163
Clinical and molecular features and therapeutic perspectives of spinal muscular atrophy with respiratory distress type 1 163
Mutational analysis of COQ2 in patients with MSA in Italy 162
CSF transplantation of a specific iPSC-derived neural stem cell subpopulation ameliorates the disease phenotype in a mouse model of spinal muscular atrophy with respiratory distress type 1 162
Totale 31.592
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Categoria #
all - tutte 180.869
article - articoli 0
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 0
Totale 180.869
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2019/20201.696 0 0 0 0 0 0 0 0 0 933 438 325
2020/20217.355 429 509 548 400 635 576 629 563 749 539 981 797
2021/20228.034 554 451 417 649 570 646 597 624 1.029 729 571 1.197
2022/20238.874 1.041 946 684 875 865 1.415 391 624 942 293 472 326
2023/20247.999 401 561 620 636 1.492 537 510 611 358 577 755 941
2024/20259.433 780 1.329 600 1.563 1.082 730 821 1.444 1.037 47 0 0
Totale 65.559