IRIS Institutional Research Information System - OPENBS Open Archive UniBS

GILIANI, SILVIA CLARA
 Distribuzione geografica
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Continente #
NA - Nord America 13.731
AS - Asia 7.267
EU - Europa 5.851
SA - Sud America 1.447
AF - Africa 178
OC - Oceania 23
Continente sconosciuto - Info sul continente non disponibili 6
Totale 28.503
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Nazione #
US - Stati Uniti d'America 13.493
SG - Singapore 2.477
CN - Cina 2.411
IT - Italia 1.205
BR - Brasile 1.199
UA - Ucraina 1.105
DE - Germania 869
HK - Hong Kong 838
VN - Vietnam 489
FI - Finlandia 487
PL - Polonia 472
GB - Regno Unito 403
FR - Francia 378
TR - Turchia 266
RU - Federazione Russa 256
BD - Bangladesh 218
IE - Irlanda 210
IN - India 176
CA - Canada 127
NL - Olanda 99
SE - Svezia 89
AR - Argentina 75
MX - Messico 72
ES - Italia 70
IQ - Iraq 55
ZA - Sudafrica 54
AT - Austria 52
JP - Giappone 52
EC - Ecuador 47
ID - Indonesia 44
CO - Colombia 35
NG - Nigeria 33
KR - Corea 29
UZ - Uzbekistan 28
BE - Belgio 26
PK - Pakistan 25
VE - Venezuela 24
PY - Paraguay 23
AU - Australia 22
SA - Arabia Saudita 22
MA - Marocco 20
CZ - Repubblica Ceca 19
CH - Svizzera 15
CL - Cile 14
IR - Iran 14
LT - Lituania 14
PE - Perù 13
TN - Tunisia 13
EG - Egitto 12
RO - Romania 11
UY - Uruguay 11
PH - Filippine 10
AZ - Azerbaigian 9
HN - Honduras 9
JM - Giamaica 9
KE - Kenya 9
KZ - Kazakistan 9
AE - Emirati Arabi Uniti 8
AL - Albania 8
DK - Danimarca 8
DZ - Algeria 8
ET - Etiopia 8
IL - Israele 8
JO - Giordania 8
KG - Kirghizistan 8
LA - Repubblica Popolare Democratica del Laos 8
NP - Nepal 8
LB - Libano 7
MU - Mauritius 7
SN - Senegal 7
GR - Grecia 6
MD - Moldavia 6
OM - Oman 6
PS - Palestinian Territory 6
EU - Europa 5
MY - Malesia 5
RS - Serbia 5
DO - Repubblica Dominicana 4
LU - Lussemburgo 4
PT - Portogallo 4
TW - Taiwan 4
BA - Bosnia-Erzegovina 3
BG - Bulgaria 3
BO - Bolivia 3
CR - Costa Rica 3
CY - Cipro 3
EE - Estonia 3
HR - Croazia 3
HU - Ungheria 3
KH - Cambogia 3
MK - Macedonia 3
PA - Panama 3
SI - Slovenia 3
TH - Thailandia 3
TT - Trinidad e Tobago 3
AM - Armenia 2
BH - Bahrain 2
CI - Costa d'Avorio 2
GA - Gabon 2
GE - Georgia 2
Totale 28.474
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Città #
Fairfield 1.464
Singapore 1.302
Ashburn 1.283
Woodbridge 1.122
Houston 871
Hong Kong 835
Jacksonville 801
Ann Arbor 597
Seattle 586
Cambridge 569
San Jose 564
Beijing 490
Wilmington 479
Warsaw 466
The Dalles 419
Chandler 407
New York 355
Princeton 350
Helsinki 269
Nanjing 259
Brescia 257
Los Angeles 241
Lauterbourg 220
Dublin 209
Istanbul 199
Munich 190
Ho Chi Minh City 179
Milan 143
Des Moines 137
Naples 110
Buffalo 108
Nanchang 105
Dearborn 103
Dallas 101
São Paulo 98
Changsha 97
Shenyang 97
Chicago 96
Hanoi 95
Moscow 93
San Francisco 93
Shanghai 85
Jinan 84
Redondo Beach 80
Turku 79
Council Bluffs 76
San Diego 68
Tianjin 67
Santa Clara 66
London 63
Hebei 59
Orem 58
Chennai 55
Jiaxing 52
Lancaster 48
Brooklyn 42
Rome 41
Tokyo 39
Frankfurt am Main 38
Nuremberg 38
Rio de Janeiro 37
Atlanta 36
Kunming 36
Stockholm 36
Denver 35
Hangzhou 34
Abuja 33
Zhengzhou 33
Manchester 32
Montreal 32
Ningbo 32
Johannesburg 31
Verona 31
Toronto 30
Haikou 29
Phoenix 29
Guangzhou 28
Poplar 28
Amsterdam 27
Charlotte 26
Dong Ket 26
Vienna 26
Baghdad 25
Boston 25
Jakarta 25
Mexico City 25
Salt Lake City 25
Brasília 24
Lanzhou 24
Boardman 23
Brussels 23
Tashkent 22
Assemini 21
Haiphong 21
Belo Horizonte 20
Düsseldorf 20
Kocaeli 20
Mumbai 20
Curitiba 19
Quito 19
Totale 18.685
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Nome #
A novel primary human immunodeficiency due to deficiency in the WASP-interacting protein WIP 388
iPSC-DERIVED NEURAL STEM CELLS CULTURED ON ENGINEERED SUBSTRATES AS AN IN VITRO MODEL FOR AICARDI-GOUTIÈRES SYNDROME 254
Aicardi-Goutieres syndrome, a rare neurological disease in children: a new autoimmune disorder? 233
Sine causa tetraparesis: A pilot study on its possible relationship with interferon signature analysis and Aicardi Goutières syndrome related genes analysis 233
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 227
Generation of induced pluripotent stem cell (iPSC) lines from a Joubert syndrome patient with compound heterozygous mutations in C5orf42 gene 220
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 211
A peptide derived from the Wiskott-Aldrich syndrome (WAS) protein-interacting protein (WIP) restores WAS protein level and actincytoskeleton reorganization in lymphocytes from patients with WAS mutations that disrupt WIP binding 210
Generation of 3 clones of induced pluripotent stem cells (iPSCs) from a patient affected by Crohn's disease 207
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 204
Selective laser melting and electron beam melting of Ti6Al4V for orthopedic applications: A comparative study on the applied building direction 203
Different molecular behavior of CD40 mutants causing hyper-IgM syndrome. 202
Eye model for floaters’ studies: production of 3D printed scaffolds 197
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 196
Genetic variation in schlafen genes in a patient with a recapitulation of the murine Elektra phenotype. 191
Altered germinal center reaction and abnormal B cell peripheral maturation in PI3KR1-mutated patients presenting with HIGM-like phenotype 185
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 185
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 184
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 183
A case of lymphoproliferative disease presenting with a clinical and immunological phenotype of common variable immunodeficiency. 182
IPSC-DERIVED NEURONS AS AN IN VITRO MODEL FOR THE STUDY OF INTERFERONOPATHIES WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT 182
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 181
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 177
First report of successful stem cell transplantation in a child with CD40 deficiency 176
Monocytes from Wiskott-Aldrich patients differentiate in functional mature dendritic cells with a defect in CD83 expression 174
Long term outcome of eight patients with type 1 Leukocyte Adhesion Deficiency (LAD-1): Not only infections, but high risk of autoimmune complications 174
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 172
Next Generation Sequencing Analysis in Early Onset Dementia Patients 172
Generation of induced pluripotent stem cells (iPSCs) from patient with Cri du Chat Syndrome 169
Adult-onset manifestation of idiopathic T-cell lymphopenia due to a heterozygous RAG1 mutation. 168
Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency 168
A novel mitochondrial tRNA(Ala) gene variant causes chronic progressive external ophthalmoplegia in a patient with Huntington disease. 167
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 166
Reduced thymic output, cell cycle abnormalities, and increasedapoptosis of T lymphocytes in patients with cartilage-hair hypoplasia 163
Cohort of Iranian Patients with Congenital Agammaglobulinemia: Mutation Analysis and Novel Gene Defects 163
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 163
Carbonization of polymer precursors substrates to direct human iPSC-derived neurons differentiation and maturation 162
Heterozygous mutation in adenosine deaminase gene in a patient with severe lymphopenia following corticosteroid treatment of autoimmune hemolytic anemia 161
A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency. 160
Whole-exome sequencing identifies tetratricopeptide repeat domain 7A (TTC7A) mutations for combined immunodeficiency with intestinal atresias 160
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 159
DISCRIMINATION AMONG MONOGENIC AND ACQUIRED DISEASE WITH INFLAMMATORY AND AUTOIMMUNE COMPONENT THROUGH THE IN VITRO STUDY OF SIGNAL TRANSDUCERS OF TYPE I INTERFERON 159
Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56bright NKG2A+++ Cells, and Yet Display Increased Degranulation and Higher Perforin Content. 155
Targeted NGS Platforms for Genetic Screening and Gene Discovery in Primary Immunodeficiencies 155
Persistent Infection with Rotavirus Vaccine Strain in Severe Combined Immunodeficiency (SCID) Child: Is Rotavirus Vaccination in SCID Children a Janus Face? 155
A novel mutation in the POLE2 gene causing combined immunodeficiency 155
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 154
E-Cadherin expression and blunted interferon response in blastic plasmacytoid dendritic cell neoplasm 154
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 153
Severe combined immunodeficiency in Serbia and Montenegro between years 1986 and 2010: a single-center experience. 152
A boy with X-linked hyper-IgM syndrome and natural killer cell deficiency. 152
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 152
Aicardi Goutieres Syndrome: New Insights on a Novel Set of Inborn Errors of Immunity 152
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 151
A systematic analysis of recombination activity and genotype-phenotype correlation in human recombination-activating gene 1 deficiency. 150
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 148
CD40 and CD40 Ligand Deficiencies 147
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 146
Different In Vitro Models of Chronic Myeloid Leukemia Show Different Characteristics: Biological Replicates Are Not Biologically Equivalent 145
Patients' induced pluripotent stem cells to model drug induced adverse events: a role in predicting thiopurine induced pancreatitis? 145
Fenotipo clinico, valutazione immunologia e analisi molecolare in pazienti affetti da trombocitemia isolata X-recessiva (XLT) 144
Toll-like receptor 3 gene polymorphisms and severity of pandemic A/H1N1/2009 influenza in otherwise healthy children. 144
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome: time to review diagnostic criteria? 144
Prenatal molecular diagnosis of Wiskott-Aldrich syndrome by direct mutation analysis 143
The role of induced pluripotent stem cells in research and therapy of primary immunodeficiencies. 143
ALTERED B AND T LYMPHOCYTE HOMEOSTASIS IN THE BONE MARROW AND THE PERIPHERY CORRELATES WITH CLINICAL FINDINGS IN COMMON VARIABLE IMMUNODEFICIENCY (CVID) 143
Intronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia. 142
Small RNAs derived from lncRNA RNase MRP have gene-silencing activity relevant to human cartilage-hair hypoplasia 142
Establishment of three iPSC lines from fibroblasts of a patient with Aicardi Goutières syndrome mutated in RNaseH2B 142
Activated Phosphoinositide 3-Kinase Delta Syndrome 1: Clinical and Immunological Data from an Italian Cohort of Patients 142
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 141
Selective laser melting and electron beam melting of Ti6Al4V for orthopedic applications: A comparative study on the applied building direction 141
Biomarkers and precision therapy for primary immunodeficiencies: an in vitro study based on induced pluripotent stem cells (iPSCs) from patients 140
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 140
Complete genomic organization of the human JAK3 gene and mutation analysis in severe combined immunodeficiency by single-strand conformation polymorphism. 138
Application of common recommendations for diagnosis and treatment of Wiskott-Aldrich syndrome and X-recessive piastrinopenia: clinical and immunological characteristics of 65 patients with WAS/XLT 138
Low WASp expression in patients with no apparent mutation in the WASP gene 137
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 136
CASE REPORT: IDENTIFICATION OF A PATIENT WITH FAMILIAR MUTATIONS AFFECTING BOTH WASP AND FOXP3 GENES 136
Generation of three isogenic induced Pluripotent Stem Cell lines (iPSCs) from fibroblasts of a patient with Aicardi Goutières Syndrome carrying a c.2471G>A dominant mutation in IFIH1 gene 135
Pathological 25 kDa C-Terminal Fragments of TDP-43 Are Present in Lymphoblastoid Cell Lines and Extracellular Vesicles from Patients Affected by Frontotemporal Lobar Degeneration and Neuronal Ceroidolipofuscinosis Carrying a GRN Mutation 134
Production of Micro-patterned Substrates to Direct Human iPSCs-derived Neural Stem Cells Orientation and Interaction 134
Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort 134
The Wiskott-Aldrich syndrome: From genotype-phenotype correlation to treatment 132
Prenatal diagnosis of JAK3 deficient SCID. 131
Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome 131
Defect of regulatory T cells in patients with Omenn syndrome 131
Transient Decrease of Circulating and Tissular Dendritic Cells in Patients With Mycobacterial Disease and With Partial Dominant IFNγR1 Deficiency 131
Differential role of nonhomologous end joining factors in the generation, DNA damage response, and myeloid differentiation of human induced pluripotent stem cells. 130
Primary immunodeficiency mutation databases 130
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 130
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 130
Severe impairment of IFN-γ and IFN-α responses in cells of a patient with a novel STAT1 splicing mutation. 130
Long-term outcome and lineage-specific chimerism in 194 Wiskott-Aldrich Syndrome patients treated by hematopoietic celltransplantation between 1980-2009: an international collaborative study. 130
Corrigendum : Targeted NGS platforms for genetic screening and gene discovery in primary immunodeficiencies (Frontiers in Immunology (2019) 10 (316) DOI: 10.3389/fimmu.2019.00316) 130
Organization of the human CD40L gene: implications for molecular defects in X chromosome-linked hyper-IgM syndrome and prenatal diagnosis. 129
Defective expression of CD40 ligand on T cells causes "X-linked immunodeficiency with hyper-IgM (HIGM1)". 129
MUTATION ANALYSIS BY A NON-RADIOACTIVE SINGLE-STRAND CONFORMATION POLYMORPHISM ASSAY IN NINE FAMILIES WITH X-LINKED SEVERE COMBINED IMMUNODEFICIENCY (SCIDX1). 129
Damaging-agent sensitivity of Artemis-deficient cell lines 129
Partial V(D)J recombination activity leads to Omenn syndrome 129
Totale 16.136
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Categoria #
all - tutte 136.210
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 136.210
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2020/2021116 0 0 0 0 0 0 0 0 0 0 0 116
2021/20221.502 95 275 44 94 14 55 98 94 90 177 130 336
2022/20231.325 205 16 33 73 149 349 12 136 189 17 66 80
2023/20241.711 88 29 152 183 86 307 70 80 412 17 50 237
2024/20254.491 16 52 57 550 396 313 358 203 555 494 892 605
2025/20269.174 837 1.124 629 1.341 905 646 1.236 430 506 734 469 317
Totale 29.002