IRIS Institutional Research Information System - OPENBS Open Archive UniBS

NOTARANGELO, Luigi Daniele
 Distribuzione geografica
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Continente #
NA - Nord America 7.861
AS - Asia 3.858
EU - Europa 2.852
SA - Sud America 747
AF - Africa 105
OC - Oceania 10
Continente sconosciuto - Info sul continente non disponibili 4
Totale 15.437
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Nazione #
US - Stati Uniti d'America 7.753
CN - Cina 1.404
SG - Singapore 1.243
UA - Ucraina 770
BR - Brasile 639
DE - Germania 515
HK - Hong Kong 447
FI - Finlandia 277
GB - Regno Unito 273
IT - Italia 240
VN - Vietnam 240
FR - Francia 207
RU - Federazione Russa 148
TR - Turchia 141
IE - Irlanda 131
IN - India 127
PL - Polonia 70
SE - Svezia 64
CA - Canada 55
BD - Bangladesh 46
IQ - Iraq 37
AR - Argentina 34
NL - Olanda 33
MX - Messico 32
ES - Italia 31
JP - Giappone 27
ZA - Sudafrica 27
NG - Nigeria 26
ID - Indonesia 25
AT - Austria 20
BE - Belgio 19
SA - Arabia Saudita 18
CL - Cile 17
PK - Pakistan 15
UZ - Uzbekistan 15
EC - Ecuador 13
IR - Iran 13
CO - Colombia 12
VE - Venezuela 12
MA - Marocco 11
AU - Australia 10
CZ - Repubblica Ceca 9
PE - Perù 9
AL - Albania 8
KE - Kenya 8
AZ - Azerbaigian 7
CH - Svizzera 7
TN - Tunisia 7
EG - Egitto 6
NP - Nepal 6
AE - Emirati Arabi Uniti 5
ET - Etiopia 5
HN - Honduras 5
JO - Giordania 5
MU - Mauritius 5
PY - Paraguay 5
UY - Uruguay 5
DO - Repubblica Dominicana 4
DZ - Algeria 4
EU - Europa 4
GR - Grecia 4
KZ - Kazakistan 4
LB - Libano 4
LT - Lituania 4
OM - Oman 4
BH - Bahrain 3
BY - Bielorussia 3
CR - Costa Rica 3
EE - Estonia 3
MY - Malesia 3
PS - Palestinian Territory 3
TT - Trinidad e Tobago 3
AM - Armenia 2
BG - Bulgaria 2
JM - Giamaica 2
KG - Kirghizistan 2
KR - Corea 2
LU - Lussemburgo 2
PT - Portogallo 2
RO - Romania 2
TH - Thailandia 2
AF - Afghanistan, Repubblica islamica di 1
AO - Angola 1
BA - Bosnia-Erzegovina 1
BB - Barbados 1
BO - Bolivia 1
BS - Bahamas 1
CY - Cipro 1
DK - Danimarca 1
GA - Gabon 1
GE - Georgia 1
HR - Croazia 1
IL - Israele 1
IS - Islanda 1
KW - Kuwait 1
LS - Lesotho 1
MD - Moldavia 1
MK - Macedonia 1
MM - Myanmar 1
PA - Panama 1
Totale 15.429
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Città #
Fairfield 948
Woodbridge 742
Ashburn 682
Singapore 660
Houston 563
Jacksonville 543
Hong Kong 446
Ann Arbor 418
Cambridge 394
Seattle 353
Chandler 304
Wilmington 291
Beijing 261
Princeton 239
The Dalles 234
Nanjing 205
San Jose 201
New York 169
Helsinki 149
Dublin 131
Los Angeles 118
Lauterbourg 109
Istanbul 107
Ho Chi Minh City 87
Nanchang 86
Munich 80
Des Moines 74
Warsaw 67
São Paulo 66
Buffalo 61
Changsha 61
Moscow 60
Jinan 58
Brescia 56
Lancaster 53
Hanoi 52
Milan 50
Shenyang 49
Hebei 46
San Diego 46
San Francisco 45
Dallas 44
London 43
Chicago 42
Dearborn 42
Shanghai 40
Redondo Beach 33
Turku 32
Tianjin 31
Zhengzhou 30
Orem 29
Jiaxing 28
Abuja 26
Council Bluffs 25
Brooklyn 23
Hangzhou 22
Santa Clara 22
Kunming 21
Nuremberg 21
Belo Horizonte 20
Chennai 20
Brussels 19
Rio de Janeiro 19
Boardman 18
Guangzhou 18
Tokyo 18
Verona 18
Kocaeli 17
Toronto 17
Johannesburg 16
Amsterdam 15
Brasília 15
Denver 15
Lanzhou 15
Manchester 15
Montreal 15
Ningbo 15
Atlanta 14
Taizhou 14
Da Nang 13
Haikou 13
Baghdad 12
Boston 12
Mumbai 12
Poplar 12
Stockholm 12
Tashkent 12
Augusta 11
Mexico City 11
Norwalk 11
Erbil 10
Frankfurt am Main 10
Leawood 10
Porto Alegre 10
Taiyuan 10
Washington 10
Goiânia 9
Haiphong 9
Pune 9
San Mateo 9
Totale 10.478
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Nome #
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 220
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 193
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
Functional Characterization Of Natural Killer Cells In Type I Leukocyte Adhesion Deficiency 188
Cytogenetic and molecular characterization of a de-novo t(2p;7p) translocation involving TNS3 and EXOC6B genes in a boy with a complex syndromic phenotype 185
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 183
Lack of specific T- and B-cell clonal expansions in multiple sclerosis patients with progressive multifocal leukoencephalopathy 183
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 183
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 180
G-CSF treatment of Severe Congenital Neutropenia reverses neutropenia but does not correct the underlying functional deficiency of the neutrophil in defending against microorganisms. 178
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 177
AIRE deficiency in thymus of 2 patients with Omenn syndrome. 177
First report of successful stem cell transplantation in a child with CD40 deficiency 176
Altered leukocyte response to CXCL12 in patients with Warts Hypogammaglobulinemia, Infections, Myelokathexis (WHIM) syndrome 175
Cerebellar involvement in warts Hypogammaglobulinemia immunodeficiency myelokathexis patients: Neuroimaging and clinical findings 174
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
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
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 165
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 162
Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency 162
“Maturazione della risposta anticorpale: dal repertorio anticorpale primario a quello secondario”. 159
Defective migration of monocyte-derived dendritic cells in LAD-1 immunodeficiency. 159
X-linked agammaglobulinemia, growth hormone deficiency and delay of growth and puberty. 158
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 157
Activity of classical and alternative pathways of complement in preterm and small for gestational age infants. 157
Pancreatitis in systemic lupus erythematosus. 157
Cellular and molecular pathogenesis of X-linked lymphoproliferative disease. 153
In vitro cell death of activated lymphocytes in Omenn's syndrome 153
Signaling via IL-2 and IL-4 in JAK3-deficient severe combined immunodeficiency lymphocytes: JAK3-dependent and independent pathways. 152
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 152
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 150
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 148
Long-term outcome following hematopoietic stem cell transplantation in Wiskott-Aldrich syndrome: collaborative study of the European Society for Immunodeficiencies and the European Group for Blood and Marrow Transplantation 147
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 146
AIRE and immunological tolerance: insights from the study of autoimmune polyendocrinopathy candidiasis and ectodermal dystrophy 146
Western Blot technique in the serological evaluation of three LAV/HTLV III-infected Italian families. 145
Leukocyte adhesion deficiency in a child with severe oral involvement. 145
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 143
Studies of the expression of the Wiskott-Aldrich syndrome protein. 141
V(D)J recombination defects in lymphocytes due to RAG mutations: severe immunodeficiency with a spectrum of clinical presentations. 141
Natural Killer cell deficiencies and severe varicella infection 140
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 139
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 139
Low WASp expression in patients with no apparent mutation in the WASP gene 136
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19 135
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 133
Auto-antibodies against type I IFNs in patients with life-threatening COVID-19 133
CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2-mediated condition 131
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 130
Reduced thymic output, increased spontaneous apoptosis and oligoclonal B cells in polyethylene glycol-adenosine deaminase-treated patients 130
Cooperative intervention to assist children with HIV-infection in Burkina Faso: The Brescia experience during the pilot phase of the ESTHER Project. 129
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 129
Isolation of Cosmid and cDNA Clones in the Region Surrounding the BTK Gene at Xq21.3-q22. 129
Partial V(D)J recombination activity leads to Omenn syndrome 128
Long-term outcome and lineage-specific chimerism in 194 Wiskott-Aldrich Syndrome patients treated by hematopoietic celltransplantation between 1980-2009: an international collaborative study. 127
Mutations of the X-linked lymphoproliferative disease gene SH2D1A mimicking common variable immunodeficiency. 124
Expansion of large lymphocyte subsets in Wiskott-Aldrich syndrome. 124
Multi-lineage analysis of X-inactivation in female carriers of genetic alterations in the Wiskott-Aldrich syndrome protein (WASP) gene locus 123
Expression of inducible Nitric Oxide Synthase (iNOS) in monocytic cells from children with infections by intracellular pathogens 123
Intrathymic restriction and peripheral expansion of the T cell repertoire in Omenn syndrome 122
Interleukin-15 (IL-15) induces IL-8 and monocyte chemotactic protein 1 production in human monocytes. 122
WASP-WIP DEFICIENCIES: NEW INSIGHTS IN WASP-WIP INTERACTION 122
CHARACTERIZATION OF AUTOANTIBODY PROFILE AMONG PATIENTS WITH PRIMARY IMMUNODEFICIENCY SECONDARY TO RAG MUTATION 120
The spleen in the Wiskott-Aldrich syndrome: histopathologic abnormalities of the white pulp correlate with the clinical phenotype of the disease. 119
Immunological and genetic bases of new primary immunodeficiencies 117
In vitro correction of JAK3-deficient severe combined immunodeficiency by retroviral-mediated gene transduction. 117
Omenn syndrome in an infant with IL7RA gene mutation 116
WASP regulates suppressor activity of human and murine CD4+CD25+FOXP3+natural regulatory T cells 114
Major histocompatibility class II molecole deficiency (bare lymphocyte sindrome): Cell type specific residual expression of classical MHC protein on dendritic cells and endothelium. 114
Jak3, severe combined immunodeficiency, and a new class of immunosuppressive drugs 113
Primary immune deficiencies unravel the molecular basis of immune response 113
Serum IgG levels and complement activity in hypogammaglobulinemic patients under substitution therapy. 113
IgG subclasses serum levels in juvenile chronic arthritis. 112
Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hot spots, effect on transcription and translation and phenotype/genotype correlation. 112
Impaired thymic output and restricted T-cell repertoire in two infants with immunodeficiency and early-onset generalized dermatitis 110
ζ Chain-associated protein of 70 kDa (ZAP70) deficiency in human subjects is associated with abnormalities of thymic stromal cells: Implications for T-cell tolerance 110
Wiskott-Aldrich syndrome. A model for defective actin reorganization, cell trafficking and synapse formation 108
Defective expression of HLA class I and CD1a molecules in a boy with a Marfan-like phenotype and deep skin ulcers. 107
CD4+ cells from patients with common variable immunodeficiency are intrinsically defective in their ability to express membrane CD40 ligand after in vitro stimulation. 107
X-linked lymphoproliferative disease. 2B4 molecules displaying inhibitory rather than activating function are responsible for the inability of natural killer cells to kill Epstein-Barr virus-infected cells 106
A new immunoperoxidase assay for lolium perenne-specific IgE in serum based on the biotin/avidin system (BAS). 105
Cutaneous CD30+ cells in children with atopic dermatitis 103
Long-Term Outcome of WHIM Syndrome in 18 Patients: High Risk of Lung Disease and HPV-Related Malignancies 102
WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function 101
Antibody responses to the SARS-CoV-2 vaccine in individuals with various inborn errors of immunity 101
IgM and IgG concentrations in the serum and secretions of children with selective IgA deficiency. 99
PTX3 genetic variations affect the risk of Pseudomonas aeruginosa airway colonization in cystic fibrosis patients 99
Genotyping for guiding drug choice in human immunodeficiency virus-infected children failing multiple antiretroviral treatment regimens [2] 98
Linker for Activation of T cells (LAT), a novel immunohistochemical marker for T cells, NK cells, mast cells, and megakaryocytes. Evaluation in normal and pathological conditions 97
Inborn Errors of Immunity With Immune Dysregulation: From Bench to Bedside. 97
SP110 REGULATES NUCLEAR ORPHAN RECEPTOR NUR77-DRIVEN APOPTOSIS IN T CELLS 96
Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Diseases: Current Status and Future Perspectives. 96
Molecular and biochemical characterization of JAK3 deficiency in a patient with severe combined immunodeficiency over 20 years after bone marrow transplantation: Implications for treatment 94
Human genetic and immunological determinants of critical COVID-19 pneumonia 94
Henoch-Schoenlein syndrome and selective IgA deficiency. 93
Ineffective expression of CD40L on cord blood T cells may contribute to poor immunoglobulin production in the newborn. 90
Characterization of T and B cell repertoire diversity in patients with RAG deficiency 90
In-utero transplantation of parental CD34 haematopoietic progenitor celsin a patient ith x-linked severe combined immunodeficiency 89
Totale 13.558
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Categoria #
all - tutte 74.364
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 74.364
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2020/2021495 0 0 0 0 0 0 0 0 0 237 199 59
2021/20221.040 60 235 12 54 21 36 73 49 42 133 96 229
2022/2023885 146 24 24 56 96 218 1 101 138 4 38 39
2023/2024908 49 12 93 87 44 161 37 40 228 7 2 148
2024/20252.046 7 9 3 279 224 156 203 65 222 135 452 291
2025/20263.874 398 614 322 646 439 269 544 161 223 258 0 0
Totale 15.663