IRIS Institutional Research Information System - OPENBS Open Archive UniBS

GILIANI, SILVIA CLARA
 Distribuzione geografica
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Continente #
NA - Nord America 13.122
AS - Asia 7.099
EU - Europa 5.690
SA - Sud America 1.446
AF - Africa 177
OC - Oceania 23
Continente sconosciuto - Info sul continente non disponibili 6
Totale 27.563
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Nazione #
US - Stati Uniti d'America 12.911
SG - Singapore 2.458
CN - Cina 2.394
BR - Brasile 1.198
UA - Ucraina 1.104
IT - Italia 1.063
DE - Germania 869
HK - Hong Kong 837
VN - Vietnam 488
FI - Finlandia 487
PL - Polonia 472
GB - Regno Unito 401
FR - Francia 377
TR - Turchia 266
RU - Federazione Russa 255
IE - Irlanda 210
IN - India 175
CA - Canada 107
NL - Olanda 91
BD - Bangladesh 90
SE - Svezia 89
AR - Argentina 75
MX - Messico 71
ES - Italia 68
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 14
CL - Cile 14
IR - Iran 14
LT - Lituania 14
PE - Perù 13
TN - Tunisia 13
EG - Egitto 11
RO - Romania 11
UY - Uruguay 11
PH - Filippine 10
AZ - Azerbaigian 9
HN - Honduras 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
JM - Giamaica 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
OM - Oman 6
PS - Palestinian Territory 6
EU - Europa 5
MY - Malesia 5
RS - Serbia 5
DO - Repubblica Dominicana 4
LU - Lussemburgo 4
MD - Moldavia 4
PT - Portogallo 4
TW - Taiwan 4
BA - Bosnia-Erzegovina 3
BG - Bulgaria 3
BO - Bolivia 3
EE - Estonia 3
HR - Croazia 3
HU - Ungheria 3
KH - Cambogia 3
MK - Macedonia 3
PA - Panama 3
TH - Thailandia 3
AM - Armenia 2
BH - Bahrain 2
CI - Costa d'Avorio 2
CR - Costa Rica 2
CY - Cipro 2
GA - Gabon 2
GE - Georgia 2
MT - Malta 2
NO - Norvegia 2
Totale 27.537
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Città #
Fairfield 1.464
Singapore 1.294
Ashburn 1.221
Woodbridge 1.122
Houston 864
Hong Kong 834
Jacksonville 801
Ann Arbor 597
Seattle 584
Cambridge 568
Beijing 488
Wilmington 478
San Jose 468
Warsaw 466
The Dalles 419
Chandler 407
Princeton 350
New York 316
Helsinki 269
Nanjing 259
Brescia 248
Los Angeles 229
Lauterbourg 220
Dublin 209
Istanbul 199
Munich 190
Ho Chi Minh City 179
Des Moines 137
Milan 133
Nanchang 105
Buffalo 103
Dearborn 103
São Paulo 98
Changsha 97
Shenyang 97
Chicago 95
Dallas 95
Hanoi 94
Moscow 92
San Francisco 89
Shanghai 85
Jinan 84
Redondo Beach 80
Turku 79
Council Bluffs 76
San Diego 68
Tianjin 67
London 63
Hebei 59
Orem 58
Santa Clara 56
Chennai 55
Jiaxing 52
Lancaster 47
Brooklyn 40
Tokyo 39
Frankfurt am Main 38
Nuremberg 38
Rio de Janeiro 37
Kunming 36
Stockholm 36
Atlanta 35
Abuja 33
Denver 33
Hangzhou 33
Zhengzhou 33
Ningbo 32
Rome 32
Johannesburg 31
Manchester 30
Haikou 29
Verona 29
Guangzhou 28
Montreal 28
Poplar 28
Amsterdam 27
Phoenix 27
Toronto 27
Dong Ket 26
Vienna 26
Baghdad 25
Jakarta 25
Salt Lake City 25
Boston 24
Brasília 24
Charlotte 24
Lanzhou 24
Mexico City 24
Boardman 23
Brussels 23
Tashkent 22
Assemini 21
Haiphong 21
Belo Horizonte 20
Düsseldorf 20
Kocaeli 20
Curitiba 19
Mumbai 19
Quito 19
Seongnam 19
Totale 18.282
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Nome #
A novel primary human immunodeficiency due to deficiency in the WASP-interacting protein WIP 384
iPSC-DERIVED NEURAL STEM CELLS CULTURED ON ENGINEERED SUBSTRATES AS AN IN VITRO MODEL FOR AICARDI-GOUTIÈRES SYNDROME 252
Aicardi-Goutieres syndrome, a rare neurological disease in children: a new autoimmune disorder? 232
Sine causa tetraparesis: A pilot study on its possible relationship with interferon signature analysis and Aicardi Goutières syndrome related genes analysis 227
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 220
Generation of induced pluripotent stem cell (iPSC) lines from a Joubert syndrome patient with compound heterozygous mutations in C5orf42 gene 216
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 209
Generation of 3 clones of induced pluripotent stem cells (iPSCs) from a patient affected by Crohn's disease 206
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 202
Different molecular behavior of CD40 mutants causing hyper-IgM syndrome. 201
Eye model for floaters’ studies: production of 3D printed scaffolds 193
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 193
Genetic variation in schlafen genes in a patient with a recapitulation of the murine Elektra phenotype. 191
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 191
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 183
Altered germinal center reaction and abnormal B cell peripheral maturation in PI3KR1-mutated patients presenting with HIGM-like phenotype 183
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 183
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 180
IPSC-DERIVED NEURONS AS AN IN VITRO MODEL FOR THE STUDY OF INTERFERONOPATHIES WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT 180
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 180
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 171
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 171
Next Generation Sequencing Analysis in Early Onset Dementia Patients 171
Long term outcome of eight patients with type 1 Leukocyte Adhesion Deficiency (LAD-1): Not only infections, but high risk of autoimmune complications 170
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 167
Generation of induced pluripotent stem cells (iPSCs) from patient with Cri du Chat Syndrome 167
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 165
A novel mitochondrial tRNA(Ala) gene variant causes chronic progressive external ophthalmoplegia in a patient with Huntington disease. 165
Cohort of Iranian Patients with Congenital Agammaglobulinemia: Mutation Analysis and Novel Gene Defects 162
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 162
A case of lymphoproliferative disease presenting with a clinical and immunological phenotype of common variable immunodeficiency. 161
A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency. 159
DISCRIMINATION AMONG MONOGENIC AND ACQUIRED DISEASE WITH INFLAMMATORY AND AUTOIMMUNE COMPONENT THROUGH THE IN VITRO STUDY OF SIGNAL TRANSDUCERS OF TYPE I INTERFERON 158
Heterozygous mutation in adenosine deaminase gene in a patient with severe lymphopenia following corticosteroid treatment of autoimmune hemolytic anemia 158
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 157
Whole-exome sequencing identifies tetratricopeptide repeat domain 7A (TTC7A) mutations for combined immunodeficiency with intestinal atresias 157
Carbonization of polymer precursors substrates to direct human iPSC-derived neurons differentiation and maturation 155
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. 154
Persistent Infection with Rotavirus Vaccine Strain in Severe Combined Immunodeficiency (SCID) Child: Is Rotavirus Vaccination in SCID Children a Janus Face? 154
A novel mutation in the POLE2 gene causing combined immunodeficiency 154
Targeted NGS Platforms for Genetic Screening and Gene Discovery in Primary Immunodeficiencies 153
Severe combined immunodeficiency in Serbia and Montenegro between years 1986 and 2010: a single-center experience. 152
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 152
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 151
A systematic analysis of recombination activity and genotype-phenotype correlation in human recombination-activating gene 1 deficiency. 150
Aicardi Goutieres Syndrome: New Insights on a Novel Set of Inborn Errors of Immunity 150
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 150
A boy with X-linked hyper-IgM syndrome and natural killer cell deficiency. 149
Reduced thymic output, cell cycle abnormalities, and increasedapoptosis of T lymphocytes in patients with cartilage-hair hypoplasia 149
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 148
CD40 and CD40 Ligand Deficiencies 147
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 146
Patients' induced pluripotent stem cells to model drug induced adverse events: a role in predicting thiopurine induced pancreatitis? 144
Prenatal molecular diagnosis of Wiskott-Aldrich syndrome by direct mutation analysis 143
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 143
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome: time to review diagnostic criteria? 143
Fenotipo clinico, valutazione immunologia e analisi molecolare in pazienti affetti da trombocitemia isolata X-recessiva (XLT) 142
The role of induced pluripotent stem cells in research and therapy of primary immunodeficiencies. 142
Intronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia. 142
ALTERED B AND T LYMPHOCYTE HOMEOSTASIS IN THE BONE MARROW AND THE PERIPHERY CORRELATES WITH CLINICAL FINDINGS IN COMMON VARIABLE IMMUNODEFICIENCY (CVID) 142
Toll-like receptor 3 gene polymorphisms and severity of pandemic A/H1N1/2009 influenza in otherwise healthy children. 142
Small RNAs derived from lncRNA RNase MRP have gene-silencing activity relevant to human cartilage-hair hypoplasia 141
Establishment of three iPSC lines from fibroblasts of a patient with Aicardi Goutières syndrome mutated in RNaseH2B 140
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 139
Biomarkers and precision therapy for primary immunodeficiencies: an in vitro study based on induced pluripotent stem cells (iPSCs) from patients 139
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 139
Different In Vitro Models of Chronic Myeloid Leukemia Show Different Characteristics: Biological Replicates Are Not Biologically Equivalent 138
Complete genomic organization of the human JAK3 gene and mutation analysis in severe combined immunodeficiency by single-strand conformation polymorphism. 138
Activated Phosphoinositide 3-Kinase Delta Syndrome 1: Clinical and Immunological Data from an Italian Cohort of Patients 137
Low WASp expression in patients with no apparent mutation in the WASP gene 136
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 135
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 133
Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort 132
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 132
CASE REPORT: IDENTIFICATION OF A PATIENT WITH FAMILIAR MUTATIONS AFFECTING BOTH WASP AND FOXP3 GENES 131
Production of Micro-patterned Substrates to Direct Human iPSCs-derived Neural Stem Cells Orientation and Interaction 131
Differential role of nonhomologous end joining factors in the generation, DNA damage response, and myeloid differentiation of human induced pluripotent stem cells. 130
Prenatal diagnosis of JAK3 deficient SCID. 130
Primary immunodeficiency mutation databases 130
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 130
The Wiskott-Aldrich syndrome: From genotype-phenotype correlation to treatment 130
Damaging-agent sensitivity of Artemis-deficient cell lines 129
Development of autologous T lymphocytes in two males with X-linked severe combined immune deficiency: molecular and cellular characterization 129
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 129
Defect of regulatory T cells in patients with Omenn syndrome 129
Transient Decrease of Circulating and Tissular Dendritic Cells in Patients With Mycobacterial Disease and With Partial Dominant IFNγR1 Deficiency 129
Organization of the human CD40L gene: implications for molecular defects in X chromosome-linked hyper-IgM syndrome and prenatal diagnosis. 128
MUTATION ANALYSIS BY A NON-RADIOACTIVE SINGLE-STRAND CONFORMATION POLYMORPHISM ASSAY IN NINE FAMILIES WITH X-LINKED SEVERE COMBINED IMMUNODEFICIENCY (SCIDX1). 128
Partial V(D)J recombination activity leads to Omenn syndrome 128
Autonomous role of Wiskott-Aldrich Syndrome platelet deficiency in inducing autoimmunity and inflammation 128
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) 128
Defective expression of CD40 ligand on T cells causes "X-linked immunodeficiency with hyper-IgM (HIGM1)". 127
Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome 127
PROSPECTIVE STUDY OF 84 PATIENTS WITH WISKOTT-ALDRICH SYNDROME AND X-RECESSIVE PIASTRINOPENIA: RESULTS OF THE APPLICATION OF COMMON PROTOCOL FOR DIAGNOSIS AND TREATMENT OF WAS/XLT 127
Homozygous DNA Ligase IV R278H Mutation in Mice Leads to Leaky Scid and Represents A Model For Human LIG4 Syndrome 127
Report of the ESID collaborative study on clinical features and molecular analysis in X-LINKED HYPER-IgM syndrome 127
Severe impairment of IFN-γ and IFN-α responses in cells of a patient with a novel STAT1 splicing mutation. 127
Totale 15.783
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Categoria #
all - tutte 128.044
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 128.044
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2020/2021714 0 0 0 0 0 0 0 0 0 316 282 116
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
2025/20268.234 837 1.124 629 1.341 905 646 1.236 430 506 580 0 0
Totale 28.062