IRIS Institutional Research Information System - OPENBS Open Archive UniBS

GILIANI, SILVIA CLARA
 Distribuzione geografica
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Continente #
NA - Nord America 11.936
AS - Asia 6.208
EU - Europa 5.066
SA - Sud America 1.368
AF - Africa 114
OC - Oceania 21
Continente sconosciuto - Info sul continente non disponibili 6
Totale 24.719
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Nazione #
US - Stati Uniti d'America 11.751
CN - Cina 2.108
SG - Singapore 2.102
BR - Brasile 1.150
UA - Ucraina 1.097
IT - Italia 850
DE - Germania 848
HK - Hong Kong 810
PL - Polonia 470
FI - Finlandia 429
VN - Vietnam 403
GB - Regno Unito 376
TR - Turchia 254
RU - Federazione Russa 253
IE - Irlanda 206
IN - India 141
FR - Francia 140
CA - Canada 93
SE - Svezia 87
BD - Bangladesh 79
NL - Olanda 72
MX - Messico 65
AR - Argentina 64
ES - Italia 61
AT - Austria 49
ZA - Sudafrica 47
EC - Ecuador 46
JP - Giappone 41
ID - Indonesia 40
IQ - Iraq 40
CO - Colombia 32
KR - Corea 27
BE - Belgio 26
UZ - Uzbekistan 22
AU - Australia 20
PY - Paraguay 20
VE - Venezuela 20
SA - Arabia Saudita 18
CZ - Repubblica Ceca 17
MA - Marocco 17
IR - Iran 14
LT - Lituania 14
PK - Pakistan 14
CH - Svizzera 12
PE - Perù 12
CL - Cile 10
EG - Egitto 10
TN - Tunisia 10
KZ - Kazakistan 9
AE - Emirati Arabi Uniti 8
JM - Giamaica 8
JO - Giordania 8
LA - Repubblica Popolare Democratica del Laos 8
UY - Uruguay 8
ET - Etiopia 7
HN - Honduras 7
IL - Israele 7
KG - Kirghizistan 7
MU - Mauritius 7
AL - Albania 6
AZ - Azerbaigian 6
GR - Grecia 6
KE - Kenya 6
OM - Oman 6
RO - Romania 6
DK - Danimarca 5
EU - Europa 5
LB - Libano 5
NP - Nepal 5
PS - Palestinian Territory 5
LU - Lussemburgo 4
MD - Moldavia 4
MY - Malesia 4
BA - Bosnia-Erzegovina 3
BG - Bulgaria 3
BO - Bolivia 3
DO - Repubblica Dominicana 3
DZ - Algeria 3
EE - Estonia 3
MK - Macedonia 3
PH - Filippine 3
PT - Portogallo 3
RS - Serbia 3
SN - Senegal 3
AM - Armenia 2
BH - Bahrain 2
HR - Croazia 2
HU - Ungheria 2
KH - Cambogia 2
NO - Norvegia 2
PA - Panama 2
PR - Porto Rico 2
TH - Thailandia 2
TT - Trinidad e Tobago 2
AF - Afghanistan, Repubblica islamica di 1
BB - Barbados 1
BN - Brunei Darussalam 1
BW - Botswana 1
BY - Bielorussia 1
CI - Costa d'Avorio 1
Totale 24.703
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Città #
Fairfield 1.464
Woodbridge 1.122
Ashburn 1.041
Singapore 979
Houston 860
Hong Kong 807
Jacksonville 801
Ann Arbor 597
Seattle 584
Cambridge 568
Beijing 485
Wilmington 478
Warsaw 464
Chandler 407
Princeton 350
New York 298
Nanjing 259
Brescia 232
Los Angeles 218
Helsinki 212
The Dalles 207
Dublin 205
Istanbul 197
Munich 190
Ho Chi Minh City 160
Des Moines 135
Nanchang 104
Dearborn 103
Buffalo 99
Changsha 97
Shenyang 97
Milan 93
Moscow 92
São Paulo 91
Chicago 88
San Francisco 87
Dallas 86
Jinan 83
Redondo Beach 80
Shanghai 79
Turku 79
Hanoi 73
Tianjin 67
San Diego 66
London 60
Hebei 59
Jiaxing 51
Lancaster 47
Santa Clara 44
Chennai 42
Brooklyn 40
Nuremberg 36
Rio de Janeiro 36
Kunming 35
Stockholm 35
Denver 32
Hangzhou 32
Ningbo 32
Atlanta 30
Tokyo 30
Haikou 29
Zhengzhou 29
Poplar 28
Johannesburg 27
Phoenix 27
Verona 27
Dong Ket 26
Guangzhou 25
Frankfurt am Main 24
Jakarta 24
Lanzhou 24
Mexico City 24
Orem 24
Toronto 24
Vienna 24
Brasília 23
Brussels 23
Charlotte 23
Montreal 23
Boardman 22
Manchester 22
Assemini 21
Boston 21
Rome 21
Salt Lake City 21
Belo Horizonte 20
Kocaeli 20
Amsterdam 19
Düsseldorf 19
Quito 19
Seongnam 19
Curitiba 17
Haiphong 17
Norwalk 17
Tashkent 17
Trieste 17
Baghdad 16
Campinas 16
Hefei 16
Romola 16
Totale 16.396
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Nome #
A novel primary human immunodeficiency due to deficiency in the WASP-interacting protein WIP 356
iPSC-DERIVED NEURAL STEM CELLS CULTURED ON ENGINEERED SUBSTRATES AS AN IN VITRO MODEL FOR AICARDI-GOUTIÈRES SYNDROME 240
Aicardi-Goutieres syndrome, a rare neurological disease in children: a new autoimmune disorder? 217
Sine causa tetraparesis: A pilot study on its possible relationship with interferon signature analysis and Aicardi Goutières syndrome related genes analysis 214
Generation of induced pluripotent stem cell (iPSC) lines from a Joubert syndrome patient with compound heterozygous mutations in C5orf42 gene 205
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 201
Generation of 3 clones of induced pluripotent stem cells (iPSCs) from a patient affected by Crohn's disease 199
Different molecular behavior of CD40 mutants causing hyper-IgM syndrome. 190
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 187
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 185
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 181
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 175
Genetic variation in schlafen genes in a patient with a recapitulation of the murine Elektra phenotype. 171
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 170
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 170
Altered germinal center reaction and abnormal B cell peripheral maturation in PI3KR1-mutated patients presenting with HIGM-like phenotype 170
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 169
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 167
Eye model for floaters’ studies: production of 3D printed scaffolds 166
First report of successful stem cell transplantation in a child with CD40 deficiency 166
Monocytes from Wiskott-Aldrich patients differentiate in functional mature dendritic cells with a defect in CD83 expression 165
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 161
Generation of induced pluripotent stem cells (iPSCs) from patient with Cri du Chat Syndrome 160
Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency 158
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 158
IPSC-DERIVED NEURONS AS AN IN VITRO MODEL FOR THE STUDY OF INTERFERONOPATHIES WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT 158
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 157
Long term outcome of eight patients with type 1 Leukocyte Adhesion Deficiency (LAD-1): Not only infections, but high risk of autoimmune complications 157
Adult-onset manifestation of idiopathic T-cell lymphopenia due to a heterozygous RAG1 mutation. 156
Cohort of Iranian Patients with Congenital Agammaglobulinemia: Mutation Analysis and Novel Gene Defects 154
Next Generation Sequencing Analysis in Early Onset Dementia Patients 154
A novel mitochondrial tRNA(Ala) gene variant causes chronic progressive external ophthalmoplegia in a patient with Huntington disease. 153
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 151
Whole-exome sequencing identifies tetratricopeptide repeat domain 7A (TTC7A) mutations for combined immunodeficiency with intestinal atresias 148
DISCRIMINATION AMONG MONOGENIC AND ACQUIRED DISEASE WITH INFLAMMATORY AND AUTOIMMUNE COMPONENT THROUGH THE IN VITRO STUDY OF SIGNAL TRANSDUCERS OF TYPE I INTERFERON 148
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. 148
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 147
A novel mutation in the POLE2 gene causing combined immunodeficiency 147
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 146
A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency. 145
Heterozygous mutation in adenosine deaminase gene in a patient with severe lymphopenia following corticosteroid treatment of autoimmune hemolytic anemia 143
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 143
Severe combined immunodeficiency in Serbia and Montenegro between years 1986 and 2010: a single-center experience. 141
A systematic analysis of recombination activity and genotype-phenotype correlation in human recombination-activating gene 1 deficiency. 140
CD40 and CD40 Ligand Deficiencies 140
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 139
Targeted NGS Platforms for Genetic Screening and Gene Discovery in Primary Immunodeficiencies 139
Persistent Infection with Rotavirus Vaccine Strain in Severe Combined Immunodeficiency (SCID) Child: Is Rotavirus Vaccination in SCID Children a Janus Face? 139
Patients' induced pluripotent stem cells to model drug induced adverse events: a role in predicting thiopurine induced pancreatitis? 138
Carbonization of polymer precursors substrates to direct human iPSC-derived neurons differentiation and maturation 138
Prenatal molecular diagnosis of Wiskott-Aldrich syndrome by direct mutation analysis 137
Aicardi Goutieres Syndrome: New Insights on a Novel Set of Inborn Errors of Immunity 137
A boy with X-linked hyper-IgM syndrome and natural killer cell deficiency. 135
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 135
The role of induced pluripotent stem cells in research and therapy of primary immunodeficiencies. 135
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 135
Small RNAs derived from lncRNA RNase MRP have gene-silencing activity relevant to human cartilage-hair hypoplasia 134
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 134
A case of lymphoproliferative disease presenting with a clinical and immunological phenotype of common variable immunodeficiency. 133
Intronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia. 133
Toll-like receptor 3 gene polymorphisms and severity of pandemic A/H1N1/2009 influenza in otherwise healthy children. 133
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome: time to review diagnostic criteria? 132
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 131
Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort 131
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 129
Fenotipo clinico, valutazione immunologia e analisi molecolare in pazienti affetti da trombocitemia isolata X-recessiva (XLT) 128
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 128
Reduced thymic output, cell cycle abnormalities, and increasedapoptosis of T lymphocytes in patients with cartilage-hair hypoplasia 127
Prenatal diagnosis of JAK3 deficient SCID. 126
Complete genomic organization of the human JAK3 gene and mutation analysis in severe combined immunodeficiency by single-strand conformation polymorphism. 126
Establishment of three iPSC lines from fibroblasts of a patient with Aicardi Goutières syndrome mutated in RNaseH2B 126
Differential role of nonhomologous end joining factors in the generation, DNA damage response, and myeloid differentiation of human induced pluripotent stem cells. 125
Primary immunodeficiency mutation databases 125
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 125
ALTERED B AND T LYMPHOCYTE HOMEOSTASIS IN THE BONE MARROW AND THE PERIPHERY CORRELATES WITH CLINICAL FINDINGS IN COMMON VARIABLE IMMUNODEFICIENCY (CVID) 125
Biomarkers and precision therapy for primary immunodeficiencies: an in vitro study based on induced pluripotent stem cells (iPSCs) from patients 125
Production of Micro-patterned Substrates to Direct Human iPSCs-derived Neural Stem Cells Orientation and Interaction 125
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 125
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 124
The Wiskott-Aldrich syndrome: From genotype-phenotype correlation to treatment 124
Defective expression of CD40 ligand on T cells causes "X-linked immunodeficiency with hyper-IgM (HIGM1)". 123
Development of autologous T lymphocytes in two males with X-linked severe combined immune deficiency: molecular and cellular characterization 123
Autonomous role of Wiskott-Aldrich Syndrome platelet deficiency in inducing autoimmunity and inflammation 123
Organization of the human CD40L gene: implications for molecular defects in X chromosome-linked hyper-IgM syndrome and prenatal diagnosis. 122
MUTATION ANALYSIS BY A NON-RADIOACTIVE SINGLE-STRAND CONFORMATION POLYMORPHISM ASSAY IN NINE FAMILIES WITH X-LINKED SEVERE COMBINED IMMUNODEFICIENCY (SCIDX1). 122
Damaging-agent sensitivity of Artemis-deficient cell lines 122
Partial V(D)J recombination activity leads to Omenn syndrome 121
Low WASp expression in patients with no apparent mutation in the WASP gene 121
Long-term outcome and lineage-specific chimerism in 194 Wiskott-Aldrich Syndrome patients treated by hematopoietic celltransplantation between 1980-2009: an international collaborative study. 121
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) 121
Immunodeficiencies due to defects of class-switch recombination. 120
Defect of regulatory T cells in patients with Omenn syndrome 120
Clinical, immunological, and molecular features of typical and atypical severe combined immunodeficiency: Report of the italian primary immunodeficiency network 120
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 119
Cytokine-mediated signalling and early defects in lymphoid development 119
Mutations of the X-linked lymphoproliferative disease gene SH2D1A mimicking common variable immunodeficiency. 118
Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome 118
Induced pluripotent stem cells: A novel frontier in the study of human primary immunodeficiencies. 118
Novel presentation of Omenn syndrome in association with aniridia. 118
Homozygous DNA Ligase IV R278H Mutation in Mice Leads to Leaky Scid and Represents A Model For Human LIG4 Syndrome 118
Totale 14.665
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Categoria #
all - tutte 122.526
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 122.526
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2020/20211.868 0 0 0 0 0 363 109 356 326 316 282 116
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
2025/20265.381 837 1.124 629 1.341 905 545 0 0 0 0 0 0
Totale 25.209