IRIS Institutional Research Information System - OPENBS Open Archive UniBS

NOTARANGELO, Luigi Daniele
 Distribuzione geografica
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Continente #
NA - Nord America 8.259
AS - Asia 3.965
EU - Europa 2.901
SA - Sud America 752
Continente sconosciuto - Info sul continente non disponibili 230
AF - Africa 108
OC - Oceania 10
Totale 16.225
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Nazione #
US - Stati Uniti d'America 8.116
CN - Cina 1.413
SG - Singapore 1.254
UA - Ucraina 770
BR - Brasile 643
DE - Germania 515
HK - Hong Kong 451
IT - Italia 281
FI - Finlandia 277
GB - Regno Unito 273
VN - Vietnam 241
FR - Francia 209
RU - Federazione Russa 149
TR - Turchia 141
IE - Irlanda 131
BD - Bangladesh 127
IN - India 127
CA - Canada 79
PL - Polonia 70
SE - Svezia 64
IQ - Iraq 37
AR - Argentina 34
NL - Olanda 33
ES - Italia 32
MX - Messico 32
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 14
IR - Iran 13
CO - Colombia 12
VE - Venezuela 12
MA - Marocco 11
AU - Australia 10
CZ - Repubblica Ceca 9
EG - Egitto 9
PE - Perù 9
AL - Albania 8
CH - Svizzera 8
JM - Giamaica 8
KE - Kenya 8
AZ - Azerbaigian 7
TN - Tunisia 7
HN - Honduras 6
NP - Nepal 6
AE - Emirati Arabi Uniti 5
ET - Etiopia 5
JO - Giordania 5
MU - Mauritius 5
PY - Paraguay 5
UY - Uruguay 5
CR - Costa Rica 4
DO - Repubblica Dominicana 4
DZ - Algeria 4
EU - Europa 4
GR - Grecia 4
KZ - Kazakistan 4
LB - Libano 4
LT - Lituania 4
MY - Malesia 4
OM - Oman 4
TT - Trinidad e Tobago 4
BH - Bahrain 3
BY - Bielorussia 3
EE - Estonia 3
PS - Palestinian Territory 3
AM - Armenia 2
BA - Bosnia-Erzegovina 2
BG - Bulgaria 2
KG - Kirghizistan 2
KR - Corea 2
LU - Lussemburgo 2
MD - Moldavia 2
PT - Portogallo 2
RO - Romania 2
RS - Serbia 2
TH - Thailandia 2
AF - Afghanistan, Repubblica islamica di 1
AO - Angola 1
BB - Barbados 1
BO - Bolivia 1
BS - Bahamas 1
CY - Cipro 1
DK - Danimarca 1
GA - Gabon 1
GD - Grenada 1
GE - Georgia 1
HR - Croazia 1
IL - Israele 1
IS - Islanda 1
KW - Kuwait 1
LS - Lesotho 1
MK - Macedonia 1
Totale 15.989
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Città #
Fairfield 948
Woodbridge 742
Ashburn 697
Singapore 664
Houston 566
Jacksonville 544
Hong Kong 450
Ann Arbor 418
Cambridge 394
Seattle 355
Chandler 304
Wilmington 291
Beijing 261
San Jose 244
Princeton 239
The Dalles 235
Nanjing 205
New York 186
Helsinki 149
Dublin 131
Los Angeles 126
Lauterbourg 109
Istanbul 107
Columbus 91
Ho Chi Minh City 87
Nanchang 86
Munich 80
Des Moines 74
São Paulo 67
Warsaw 67
Buffalo 63
Changsha 61
Moscow 61
Jinan 58
Brescia 57
Lancaster 53
Hanoi 52
Milan 50
Dallas 49
Shenyang 49
Hebei 46
San Diego 46
San Francisco 46
Council Bluffs 45
Chicago 43
London 43
Dearborn 42
Shanghai 40
Redondo Beach 33
Turku 32
Tianjin 31
Zhengzhou 31
Orem 29
Jiaxing 28
Santa Clara 27
Abuja 26
Brooklyn 26
Hangzhou 23
Toronto 22
Kunming 21
Nuremberg 21
Belo Horizonte 20
Chennai 20
Brussels 19
Rio de Janeiro 19
Verona 19
Boardman 18
Guangzhou 18
Tokyo 18
Denver 17
Kocaeli 17
Montreal 17
Phoenix 17
Johannesburg 16
Amsterdam 15
Atlanta 15
Brasília 15
Lanzhou 15
Manchester 15
Ningbo 15
Taizhou 14
Da Nang 13
Haikou 13
Baghdad 12
Boston 12
Mumbai 12
Poplar 12
Rome 12
Stockholm 12
Tashkent 12
Washington 12
Augusta 11
Mexico City 11
Norwalk 11
Erbil 10
Frankfurt am Main 10
Leawood 10
Porto Alegre 10
Taiyuan 10
Charlotte 9
Totale 10.724
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Nome #
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 229
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 226
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 197
Functional Characterization Of Natural Killer Cells In Type I Leukocyte Adhesion Deficiency 190
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 189
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 187
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 186
Lack of specific T- and B-cell clonal expansions in multiple sclerosis patients with progressive multifocal leukoencephalopathy 185
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 185
G-CSF treatment of Severe Congenital Neutropenia reverses neutropenia but does not correct the underlying functional deficiency of the neutrophil in defending against microorganisms. 184
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 182
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 180
AIRE deficiency in thymus of 2 patients with Omenn syndrome. 180
Altered leukocyte response to CXCL12 in patients with Warts Hypogammaglobulinemia, Infections, Myelokathexis (WHIM) syndrome 179
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 176
First report of successful stem cell transplantation in a child with CD40 deficiency 176
Cerebellar involvement in warts Hypogammaglobulinemia immunodeficiency myelokathexis patients: Neuroimaging and clinical findings 176
Monocytes from Wiskott-Aldrich patients differentiate in functional mature dendritic cells with a defect in CD83 expression 175
Adult-onset manifestation of idiopathic T-cell lymphopenia due to a heterozygous RAG1 mutation. 170
Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency 169
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 168
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 167
Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency 164
“Maturazione della risposta anticorpale: dal repertorio anticorpale primario a quello secondario”. 162
Activity of classical and alternative pathways of complement in preterm and small for gestational age infants. 162
X-linked agammaglobulinemia, growth hormone deficiency and delay of growth and puberty. 162
Defective migration of monocyte-derived dendritic cells in LAD-1 immunodeficiency. 161
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 160
Pancreatitis in systemic lupus erythematosus. 160
In vitro cell death of activated lymphocytes in Omenn's syndrome 158
Signaling via IL-2 and IL-4 in JAK3-deficient severe combined immunodeficiency lymphocytes: JAK3-dependent and independent pathways. 157
Cellular and molecular pathogenesis of X-linked lymphoproliferative disease. 157
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 156
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 154
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 154
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 152
AIRE and immunological tolerance: insights from the study of autoimmune polyendocrinopathy candidiasis and ectodermal dystrophy 150
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 149
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 149
V(D)J recombination defects in lymphocytes due to RAG mutations: severe immunodeficiency with a spectrum of clinical presentations. 147
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 147
Leukocyte adhesion deficiency in a child with severe oral involvement. 147
Western Blot technique in the serological evaluation of three LAV/HTLV III-infected Italian families. 146
Natural Killer cell deficiencies and severe varicella infection 144
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 144
Studies of the expression of the Wiskott-Aldrich syndrome protein. 142
Reduced thymic output, increased spontaneous apoptosis and oligoclonal B cells in polyethylene glycol-adenosine deaminase-treated patients 140
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19 140
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 139
Low WASp expression in patients with no apparent mutation in the WASP gene 137
Auto-antibodies against type I IFNs in patients with life-threatening COVID-19 137
CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2-mediated condition 134
Cooperative intervention to assist children with HIV-infection in Burkina Faso: The Brescia experience during the pilot phase of the ESTHER Project. 133
Isolation of Cosmid and cDNA Clones in the Region Surrounding the BTK Gene at Xq21.3-q22. 132
Partial V(D)J recombination activity leads to Omenn syndrome 131
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 131
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 131
Long-term outcome and lineage-specific chimerism in 194 Wiskott-Aldrich Syndrome patients treated by hematopoietic celltransplantation between 1980-2009: an international collaborative study. 131
Expression of inducible Nitric Oxide Synthase (iNOS) in monocytic cells from children with infections by intracellular pathogens 128
Multi-lineage analysis of X-inactivation in female carriers of genetic alterations in the Wiskott-Aldrich syndrome protein (WASP) gene locus 127
WASP-WIP DEFICIENCIES: NEW INSIGHTS IN WASP-WIP INTERACTION 126
Expansion of large lymphocyte subsets in Wiskott-Aldrich syndrome. 126
Interleukin-15 (IL-15) induces IL-8 and monocyte chemotactic protein 1 production in human monocytes. 125
Mutations of the X-linked lymphoproliferative disease gene SH2D1A mimicking common variable immunodeficiency. 125
CHARACTERIZATION OF AUTOANTIBODY PROFILE AMONG PATIENTS WITH PRIMARY IMMUNODEFICIENCY SECONDARY TO RAG MUTATION 125
Intrathymic restriction and peripheral expansion of the T cell repertoire in Omenn syndrome 124
In vitro correction of JAK3-deficient severe combined immunodeficiency by retroviral-mediated gene transduction. 123
The spleen in the Wiskott-Aldrich syndrome: histopathologic abnormalities of the white pulp correlate with the clinical phenotype of the disease. 122
WASP regulates suppressor activity of human and murine CD4+CD25+FOXP3+natural regulatory T cells 120
Omenn syndrome in an infant with IL7RA gene mutation 119
Immunological and genetic bases of new primary immunodeficiencies 118
In-utero transplantation of parental CD34 haematopoietic progenitor celsin a patient ith x-linked severe combined immunodeficiency 117
Jak3, severe combined immunodeficiency, and a new class of immunosuppressive drugs 117
IgG subclasses serum levels in juvenile chronic arthritis. 116
Primary immune deficiencies unravel the molecular basis of immune response 115
Major histocompatibility class II molecole deficiency (bare lymphocyte sindrome): Cell type specific residual expression of classical MHC protein on dendritic cells and endothelium. 115
Serum IgG levels and complement activity in hypogammaglobulinemic patients under substitution therapy. 115
Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hot spots, effect on transcription and translation and phenotype/genotype correlation. 115
CD4+ cells from patients with common variable immunodeficiency are intrinsically defective in their ability to express membrane CD40 ligand after in vitro stimulation. 113
Impaired thymic output and restricted T-cell repertoire in two infants with immunodeficiency and early-onset generalized dermatitis 112
Wiskott-Aldrich syndrome. A model for defective actin reorganization, cell trafficking and synapse formation 110
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 109
Defective expression of HLA class I and CD1a molecules in a boy with a Marfan-like phenotype and deep skin ulcers. 109
A new immunoperoxidase assay for lolium perenne-specific IgE in serum based on the biotin/avidin system (BAS). 109
Long-Term Outcome of WHIM Syndrome in 18 Patients: High Risk of Lung Disease and HPV-Related Malignancies 106
Antibody responses to the SARS-CoV-2 vaccine in individuals with various inborn errors of immunity 105
WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function 104
Cutaneous CD30+ cells in children with atopic dermatitis 103
IgM and IgG concentrations in the serum and secretions of children with selective IgA deficiency. 102
Genotyping for guiding drug choice in human immunodeficiency virus-infected children failing multiple antiretroviral treatment regimens [2] 102
PTX3 genetic variations affect the risk of Pseudomonas aeruginosa airway colonization in cystic fibrosis patients 102
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 99
Human genetic and immunological determinants of critical COVID-19 pneumonia 99
SP110 REGULATES NUCLEAR ORPHAN RECEPTOR NUR77-DRIVEN APOPTOSIS IN T CELLS 98
Henoch-Schoenlein syndrome and selective IgA deficiency. 98
Inborn Errors of Immunity With Immune Dysregulation: From Bench to Bedside. 98
Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Diseases: Current Status and Future Perspectives. 96
Characterization of T and B cell repertoire diversity in patients with RAG deficiency 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
Lack of iNKT Cells and Defects on Differentiation of Dendritic Cells in Patients with Hermansky-Pudlak Type 2 Syndrome (HPS2) 93
Totale 14.031
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Categoria #
all - tutte 81.249
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 81.249
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2021/2022980 0 235 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
2025/20264.180 398 614 322 646 439 269 544 161 223 318 120 126
2026/2027256 193 63 0 0 0 0 0 0 0 0 0 0
Totale 16.225