IRIS Institutional Research Information System - OPENBS Open Archive UniBS

GILIANI, SILVIA CLARA
 Distribuzione geografica
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Continente #
NA - Nord America 9.135
EU - Europa 3.543
AS - Asia 1.899
OC - Oceania 18
AF - Africa 11
SA - Sud America 8
Continente sconosciuto - Info sul continente non disponibili 5
Totale 14.619
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Nazione #
US - Stati Uniti d'America 9.121
CN - Cina 1.336
UA - Ucraina 1.057
DE - Germania 554
IT - Italia 491
PL - Polonia 421
HK - Hong Kong 387
FI - Finlandia 314
GB - Regno Unito 246
IE - Irlanda 198
FR - Francia 98
SE - Svezia 47
IN - India 38
SG - Singapore 32
TR - Turchia 30
VN - Vietnam 27
NL - Olanda 25
KR - Corea 24
RU - Federazione Russa 23
ES - Italia 22
BE - Belgio 21
AU - Australia 18
CA - Canada 14
JP - Giappone 8
IR - Iran 7
MU - Mauritius 7
BR - Brasile 5
EU - Europa 5
RO - Romania 5
CH - Svizzera 4
KZ - Kazakistan 3
LA - Repubblica Popolare Democratica del Laos 3
LU - Lussemburgo 3
DK - Danimarca 2
GR - Grecia 2
MA - Marocco 2
PE - Perù 2
TH - Thailandia 2
AT - Austria 1
BA - Bosnia-Erzegovina 1
BN - Brunei Darussalam 1
EE - Estonia 1
EG - Egitto 1
FK - Isole Falkland (Malvinas) 1
HR - Croazia 1
HU - Ungheria 1
IL - Israele 1
IS - Islanda 1
MD - Moldavia 1
MK - Macedonia 1
RS - Serbia 1
SC - Seychelles 1
SK - Slovacchia (Repubblica Slovacca) 1
Totale 14.619
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Città #
Fairfield 1.464
Woodbridge 1.122
Houston 834
Jacksonville 799
Ashburn 672
Ann Arbor 597
Seattle 570
Cambridge 568
Wilmington 471
Warsaw 421
Chandler 409
Hong Kong 386
Princeton 352
Nanjing 258
Beijing 227
Dublin 198
New York 195
Helsinki 188
Brescia 168
Nanchang 104
Dearborn 103
Shenyang 95
Changsha 88
Jinan 80
Des Moines 75
San Diego 65
Tianjin 62
Hebei 59
Milan 56
Jiaxing 51
Shanghai 51
Lancaster 47
London 39
Kunming 34
Ningbo 31
Haikou 28
Hangzhou 28
San Francisco 27
Verona 27
Dong Ket 26
Zhengzhou 24
Lanzhou 22
Assemini 21
Kocaeli 20
Seongnam 19
Brussels 18
Norwalk 16
Guangzhou 15
Redwood City 14
Taiyuan 14
Hefei 12
Rome 12
Taizhou 12
Kilburn 11
Boardman 10
New Bedfont 9
Toronto 9
Arezzo 8
Trieste 7
Changchun 6
Chiyoda-ku 6
Fuzhou 6
Leawood 6
Melbourne 6
Pune 6
Redmond 6
Stuttgart 6
Acton 5
Ardabil 5
Casoria 5
Mumbai 5
Orange 5
Sydney 5
Adelaide 4
Amsterdam 4
Augusta 4
Cantù 4
Florence 4
Gunzenhausen 4
Murcia 4
Napoli 4
North York 4
Nürnberg 4
Prescot 4
Santiago De Compostela 4
Southwark 4
Aubervilliers 3
Auburn Hills 3
Barcelona 3
Brooklyn 3
Chiswick 3
Frankfurt am Main 3
Islington 3
Lappeenranta 3
Lincoln 3
Los Angeles 3
Madone 3
Mannheim 3
Neustadt 3
Phoenix 3
Totale 11.520
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Nome #
A novel primary human immunodeficiency due to deficiency in the WASP-interacting protein WIP 284
iPSC-DERIVED NEURAL STEM CELLS CULTURED ON ENGINEERED SUBSTRATES AS AN IN VITRO MODEL FOR AICARDI-GOUTIÈRES SYNDROME 173
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 160
Aicardi-Goutieres syndrome, a rare neurological disease in children: a new autoimmune disorder? 159
Sine causa tetraparesis: A pilot study on its possible relationship with interferon signature analysis and Aicardi Goutières syndrome related genes analysis 152
Different molecular behavior of CD40 mutants causing hyper-IgM syndrome. 150
Generation of 3 clones of induced pluripotent stem cells (iPSCs) from a patient affected by Crohn's disease 144
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 135
Genetic variation in schlafen genes in a patient with a recapitulation of the murine Elektra phenotype. 134
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 131
CRI DU CHAT INDUCED PLURIPOTENT STEM CELLS: NEW FRONTIERS IN DISEASE UNDERSTANDING 130
Monocytes from Wiskott-Aldrich patients differentiate in functional mature dendritic cells with a defect in CD83 expression 127
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 126
Next Generation Sequencing Analysis in Early Onset Dementia Patients 121
Generation of induced pluripotent stem cells (iPSCs) from patient with Cri du Chat Syndrome 120
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 117
Whole-exome sequencing identifies tetratricopeptide repeat domain 7A (TTC7A) mutations for combined immunodeficiency with intestinal atresias 116
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 115
Altered germinal center reaction and abnormal B cell peripheral maturation in PI3KR1-mutated patients presenting with HIGM-like phenotype 115
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 115
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 114
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 113
CD40 and CD40 Ligand Deficiencies 113
Cohort of Iranian Patients with Congenital Agammaglobulinemia: Mutation Analysis and Novel Gene Defects 112
Prenatal molecular diagnosis of Wiskott-Aldrich syndrome by direct mutation analysis 111
A novel mitochondrial tRNA(Ala) gene variant causes chronic progressive external ophthalmoplegia in a patient with Huntington disease. 109
IPSC-DERIVED NEURONS AS AN IN VITRO MODEL FOR THE STUDY OF INTERFERONOPATHIES WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT 109
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 108
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 108
Adult-onset manifestation of idiopathic T-cell lymphopenia due to a heterozygous RAG1 mutation. 107
Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency 107
Severe combined immunodeficiency in Serbia and Montenegro between years 1986 and 2010: a single-center experience. 106
Small RNAs derived from lncRNA RNase MRP have gene-silencing activity relevant to human cartilage-hair hypoplasia 106
Patients' induced pluripotent stem cells to model drug induced adverse events: a role in predicting thiopurine induced pancreatitis? 106
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. 105
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 105
Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort 104
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome: time to review diagnostic criteria? 104
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 103
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 103
Toll-like receptor 3 gene polymorphisms and severity of pandemic A/H1N1/2009 influenza in otherwise healthy children. 103
First report of successful stem cell transplantation in a child with CD40 deficiency 103
Development of autologous T lymphocytes in two males with X-linked severe combined immune deficiency: molecular and cellular characterization 102
Complete genomic organization of the human JAK3 gene and mutation analysis in severe combined immunodeficiency by single-strand conformation polymorphism. 102
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 102
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 102
Differential role of nonhomologous end joining factors in the generation, DNA damage response, and myeloid differentiation of human induced pluripotent stem cells. 101
A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency. 101
A systematic analysis of recombination activity and genotype-phenotype correlation in human recombination-activating gene 1 deficiency. 100
Production of Micro-patterned Substrates to Direct Human iPSCs-derived Neural Stem Cells Orientation and Interaction 99
Mutations of the X-linked lymphoproliferative disease gene SH2D1A mimicking common variable immunodeficiency. 97
Long term outcome of eight patients with type 1 Leukocyte Adhesion Deficiency (LAD-1): Not only infections, but high risk of autoimmune complications 97
A novel mutation in the POLE2 gene causing combined immunodeficiency 97
Organization of the human CD40L gene: implications for molecular defects in X chromosome-linked hyper-IgM syndrome and prenatal diagnosis. 96
Primary immunodeficiency mutation databases 96
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 96
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 96
Defect of regulatory T cells in patients with Omenn syndrome 96
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 95
Prenatal diagnosis of JAK3 deficient SCID. 94
The role of induced pluripotent stem cells in research and therapy of primary immunodeficiencies. 94
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 94
Autonomous role of Wiskott-Aldrich Syndrome platelet deficiency in inducing autoimmunity and inflammation 93
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 92
Partial V(D)J recombination activity leads to Omenn syndrome 92
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 92
The Wiskott-Aldrich syndrome: From genotype-phenotype correlation to treatment 92
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 92
Persistent Infection with Rotavirus Vaccine Strain in Severe Combined Immunodeficiency (SCID) Child: Is Rotavirus Vaccination in SCID Children a Janus Face? 92
Ineffective expression of CD40 ligand on cord blood T cells may contribute to poor immunoglobulin production in the newborn. 91
Immunodeficiencies due to defects of class-switch recombination. 91
Intronic SH2D1A mutation with impaired SAP expression and agammaglobulinemia. 91
A boy with X-linked hyper-IgM syndrome and natural killer cell deficiency. 90
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 90
Induced pluripotent stem cells: A novel frontier in the study of human primary immunodeficiencies. 90
Targeted NGS Platforms for Genetic Screening and Gene Discovery in Primary Immunodeficiencies 90
Clinical, immunological, and molecular features of typical and atypical severe combined immunodeficiency: Report of the italian primary immunodeficiency network 90
Defective expression of CD40 ligand on T cells causes "X-linked immunodeficiency with hyper-IgM (HIGM1)". 89
Damaging-agent sensitivity of Artemis-deficient cell lines 89
Cytokine-mediated signalling and early defects in lymphoid development 89
The genomic organization of the human transcription factor 3 (TFE3) gene. 88
Single-center analysis of long-term outcome after hematopoietic cell transplantation in children with congenital severe T cell immunodeficiency. 88
Carbonization of polymer precursors substrates to direct human iPSC-derived neurons differentiation and maturation 88
Omenn syndrome in an infant with IL7RA gene mutation 86
Homozygous DNA Ligase IV R278H Mutation in Mice Leads to Leaky Scid and Represents A Model For Human LIG4 Syndrome 86
DISCRIMINATION AMONG MONOGENIC AND ACQUIRED DISEASE WITH INFLAMMATORY AND AUTOIMMUNE COMPONENT THROUGH THE IN VITRO STUDY OF SIGNAL TRANSDUCERS OF TYPE I INTERFERON 86
Reduced thymic output, cell cycle abnormalities, and increasedapoptosis of T lymphocytes in patients with cartilage-hair hypoplasia 85
Heterozygous mutation in adenosine deaminase gene in a patient with severe lymphopenia following corticosteroid treatment of autoimmune hemolytic anemia 85
Mutation analysis in Wiskott Aldrich syndrome on chorionic villus DNA 84
MECHANISMS OF PRIMARY IMMUNODEFICIENCIES: FROM BED-SIDE TO BENCH AND BACK 84
Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome 84
Novel presentation of Omenn syndrome in association with aniridia. 84
MUTATION ANALYSIS BY A NON-RADIOACTIVE SINGLE-STRAND CONFORMATION POLYMORPHISM ASSAY IN NINE FAMILIES WITH X-LINKED SEVERE COMBINED IMMUNODEFICIENCY (SCIDX1). 83
Functional analysis of naturally occurring DCLRE1C mutations and correlation with the clinical phenotype of ARTEMIS deficiency 81
Report of the ESID collaborative study on clinical features and molecular analysis in X-LINKED HYPER-IgM syndrome 80
Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hot spots, effect on transcription and translation and phenotype/genotype correlation. 79
A case of lymphoproliferative disease presenting with a clinical and immunological phenotype of common variable immunodeficiency. 77
Aicardi Goutieres Syndrome: New Insights on a Novel Set of Inborn Errors of Immunity 77
Severe impairment of IFN-γ and IFN-α responses in cells of a patient with a novel STAT1 splicing mutation. 77
Selective laser melting and electron beam melting of Ti6Al4V for orthopedic applications: A comparative study on the applied building direction 77
Totale 10.434
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Categoria #
all - tutte 67.651
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 67.651
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2018/2019982 0 0 0 0 0 0 0 0 0 197 418 367
2019/20204.352 568 123 150 502 257 480 451 465 288 703 121 244
2020/20212.847 57 300 99 343 180 363 109 356 326 316 282 116
2021/20221.503 95 275 44 94 14 55 98 94 90 177 130 337
2022/20231.332 206 16 33 73 151 350 12 137 191 17 66 80
2023/20241.430 89 29 154 184 86 307 70 80 414 17 0 0
Totale 15.064