IRIS Institutional Research Information System - OPENBS Open Archive UniBS

NOTARANGELO, Luigi Daniele
 Distribuzione geografica
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Continente #
NA - Nord America 6.210
EU - Europa 2.226
AS - Asia 1.757
OC - Oceania 10
AF - Africa 6
Continente sconosciuto - Info sul continente non disponibili 4
SA - Sud America 3
Totale 10.216
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Nazione #
US - Stati Uniti d'America 6.193
CN - Cina 910
UA - Ucraina 749
DE - Germania 406
SG - Singapore 399
HK - Hong Kong 221
FI - Finlandia 219
GB - Regno Unito 204
IT - Italia 158
RU - Federazione Russa 129
IE - Irlanda 128
TR - Turchia 126
FR - Francia 73
IN - India 64
SE - Svezia 50
PL - Polonia 47
BE - Belgio 19
NL - Olanda 19
CA - Canada 14
IR - Iran 13
AU - Australia 10
JP - Giappone 8
CZ - Repubblica Ceca 7
MU - Mauritius 5
EU - Europa 4
VN - Vietnam 4
MX - Messico 3
BD - Bangladesh 2
CH - Svizzera 2
EE - Estonia 2
ES - Italia 2
GR - Grecia 2
ID - Indonesia 2
KZ - Kazakistan 2
LT - Lituania 2
LU - Lussemburgo 2
PE - Perù 2
TH - Thailandia 2
AL - Albania 1
AT - Austria 1
BA - Bosnia-Erzegovina 1
BR - Brasile 1
IL - Israele 1
IS - Islanda 1
KR - Corea 1
MM - Myanmar 1
RO - Romania 1
SA - Arabia Saudita 1
SK - Slovacchia (Repubblica Slovacca) 1
SL - Sierra Leone 1
Totale 10.216
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Città #
Fairfield 950
Woodbridge 742
Houston 557
Jacksonville 542
Ashburn 450
Ann Arbor 418
Cambridge 395
Seattle 342
Singapore 324
Chandler 304
Wilmington 293
Princeton 241
Hong Kong 221
Nanjing 205
Beijing 143
Dublin 128
Helsinki 124
New York 111
Istanbul 105
Nanchang 84
Des Moines 63
Changsha 58
Moscow 58
Jinan 54
Lancaster 51
Shenyang 48
San Diego 47
Warsaw 47
Hebei 46
Brescia 45
Dearborn 42
Shanghai 33
Milan 32
London 31
Tianjin 30
Jiaxing 28
Zhengzhou 27
San Francisco 20
Brussels 19
Boardman 18
Kunming 18
Hangzhou 17
Kocaeli 17
Verona 16
Ningbo 15
Lanzhou 14
Taizhou 14
Guangzhou 13
Haikou 13
Munich 13
Augusta 11
Toronto 11
Leawood 10
Norwalk 10
Los Angeles 9
San Mateo 9
Taiyuan 9
Ardabil 8
Washington 8
Brno 7
Changchun 7
Pune 7
Chiyoda-ku 6
New Bedfont 6
Falkenstein 5
Islington 5
Kilburn 5
Nürnberg 5
Acton 4
Chiswick 4
Dong Ket 4
Melbourne 4
Mumbai 4
Amsterdam 3
Aubervilliers 3
Gunzenhausen 3
Orange 3
Prescot 3
Rome 3
Sydney 3
Almaty 2
Bangalore 2
Bangkok 2
Barcelona 2
Canberra 2
Cavriago 2
Chicago 2
Curtatone 2
Florence 2
Fuzhou 2
Hefei 2
Hesperange 2
Jakarta 2
Lima 2
Mexico City 2
Parma 2
Philadelphia 2
Providence 2
Redmond 2
Redwood City 2
Totale 7.845
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Nome #
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 165
Functional Characterization Of Natural Killer Cells In Type I Leukocyte Adhesion Deficiency 146
Lack of specific T- and B-cell clonal expansions in multiple sclerosis patients with progressive multifocal leukoencephalopathy 144
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 139
Monocytes from Wiskott-Aldrich patients differentiate in functional mature dendritic cells with a defect in CD83 expression 134
Pancreatitis in systemic lupus erythematosus. 133
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 131
X-linked agammaglobulinemia, growth hormone deficiency and delay of growth and puberty. 127
AIRE deficiency in thymus of 2 patients with Omenn syndrome. 126
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 124
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 124
Signaling via IL-2 and IL-4 in JAK3-deficient severe combined immunodeficiency lymphocytes: JAK3-dependent and independent pathways. 123
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 123
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 122
Altered leukocyte response to CXCL12 in patients with Warts Hypogammaglobulinemia, Infections, Myelokathexis (WHIM) syndrome 121
G-CSF treatment of Severe Congenital Neutropenia reverses neutropenia but does not correct the underlying functional deficiency of the neutrophil in defending against microorganisms. 121
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 121
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 118
In vitro cell death of activated lymphocytes in Omenn's syndrome 116
Western Blot technique in the serological evaluation of three LAV/HTLV III-infected Italian families. 115
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 114
Adult-onset manifestation of idiopathic T-cell lymphopenia due to a heterozygous RAG1 mutation. 114
First report of successful stem cell transplantation in a child with CD40 deficiency 114
Cellular and molecular pathogenesis of X-linked lymphoproliferative disease. 112
Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency 112
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 111
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 111
Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency 110
V(D)J recombination defects in lymphocytes due to RAG mutations: severe immunodeficiency with a spectrum of clinical presentations. 109
Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency 109
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 108
Natural Killer cell deficiencies and severe varicella infection 108
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 107
Activity of classical and alternative pathways of complement in preterm and small for gestational age infants. 107
Studies of the expression of the Wiskott-Aldrich syndrome protein. 106
Clinical and molecular features of X-linked hyper IgM syndrome – An experience from North India 106
Defective migration of monocyte-derived dendritic cells in LAD-1 immunodeficiency. 105
Structural and functional basis for JAK3-deficient severe combined immunodeficiency. 104
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 104
Leukocyte adhesion deficiency in a child with severe oral involvement. 104
Mutations of the X-linked lymphoproliferative disease gene SH2D1A mimicking common variable immunodeficiency. 102
Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations. 101
Mutations of Jak-3 gene in patients with autosomal severe combined immune deficiency (SCID). 100
AIRE and immunological tolerance: insights from the study of autoimmune polyendocrinopathy candidiasis and ectodermal dystrophy 100
CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2-mediated condition 100
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 100
Partial V(D)J recombination activity leads to Omenn syndrome 97
Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. 97
Reduced thymic output, increased spontaneous apoptosis and oligoclonal B cells in polyethylene glycol-adenosine deaminase-treated patients 97
“Maturazione della risposta anticorpale: dal repertorio anticorpale primario a quello secondario”. 96
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 95
The spleen in the Wiskott-Aldrich syndrome: histopathologic abnormalities of the white pulp correlate with the clinical phenotype of the disease. 94
Interleukin-15 (IL-15) induces IL-8 and monocyte chemotactic protein 1 production in human monocytes. 93
Cerebellar involvement in warts Hypogammaglobulinemia immunodeficiency myelokathexis patients: Neuroimaging and clinical findings 92
Omenn syndrome in an infant with IL7RA gene mutation 90
Defective expression of HLA class I and CD1a molecules in a boy with a Marfan-like phenotype and deep skin ulcers. 89
Isolation of Cosmid and cDNA Clones in the Region Surrounding the BTK Gene at Xq21.3-q22. 89
Intrathymic restriction and peripheral expansion of the T cell repertoire in Omenn syndrome 88
Jak3, severe combined immunodeficiency, and a new class of immunosuppressive drugs 87
Immunological and genetic bases of new primary immunodeficiencies 87
In vitro correction of JAK3-deficient severe combined immunodeficiency by retroviral-mediated gene transduction. 86
Impaired thymic output and restricted T-cell repertoire in two infants with immunodeficiency and early-onset generalized dermatitis 84
Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hot spots, effect on transcription and translation and phenotype/genotype correlation. 84
Wiskott-Aldrich syndrome. A model for defective actin reorganization, cell trafficking and synapse formation 83
Expansion of large lymphocyte subsets in Wiskott-Aldrich syndrome. 81
Low WASp expression in patients with no apparent mutation in the WASP gene 81
WASP regulates suppressor activity of human and murine CD4+CD25+FOXP3+natural regulatory T cells 80
Long-term outcome and lineage-specific chimerism in 194 Wiskott-Aldrich Syndrome patients treated by hematopoietic celltransplantation between 1980-2009: an international collaborative study. 80
Expression of inducible Nitric Oxide Synthase (iNOS) in monocytic cells from children with infections by intracellular pathogens 78
Cooperative intervention to assist children with HIV-infection in Burkina Faso: The Brescia experience during the pilot phase of the ESTHER Project. 77
IgG subclasses serum levels in juvenile chronic arthritis. 77
Cutaneous CD30+ cells in children with atopic dermatitis 77
WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function 76
WASP-WIP DEFICIENCIES: NEW INSIGHTS IN WASP-WIP INTERACTION 74
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 73
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 72
IgM and IgG concentrations in the serum and secretions of children with selective IgA deficiency. 72
Serum IgG levels and complement activity in hypogammaglobulinemic patients under substitution therapy. 70
CHARACTERIZATION OF AUTOANTIBODY PROFILE AMONG PATIENTS WITH PRIMARY IMMUNODEFICIENCY SECONDARY TO RAG MUTATION 69
Multi-lineage analysis of X-inactivation in female carriers of genetic alterations in the Wiskott-Aldrich syndrome protein (WASP) gene locus 68
Primary immune deficiencies unravel the molecular basis of immune response 67
Characterization of nine novel mutations in the CD40 ligand gene in patients with X-linked hiper IgM syndrome of various ancestry. 65
A new immunoperoxidase assay for lolium perenne-specific IgE in serum based on the biotin/avidin system (BAS). 64
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 63
Ineffective expression of CD40L on cord blood T cells may contribute to poor immunoglobulin production in the newborn. 62
CD4+ cells from patients with common variable immunodeficiency are intrinsically defective in their ability to express membrane CD40 ligand after in vitro stimulation. 62
SP110 REGULATES NUCLEAR ORPHAN RECEPTOR NUR77-DRIVEN APOPTOSIS IN T CELLS 61
Auto-antibodies against type I IFNs in patients with life-threatening COVID-19 60
Henoch-Schoenlein syndrome and selective IgA deficiency. 59
Working Model of home Care for Families with HIV infectes children. 58
Inborn Errors of Immunity With Immune Dysregulation: From Bench to Bedside. 57
Major histocompatibility class II molecole deficiency (bare lymphocyte sindrome): Cell type specific residual expression of classical MHC protein on dendritic cells and endothelium. 56
Lack of iNKT Cells and Defects on Differentiation of Dendritic Cells in Patients with Hermansky-Pudlak Type 2 Syndrome (HPS2) 56
Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Diseases: Current Status and Future Perspectives. 56
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19 55
Monocytes from Wiskott-Aldrich patients desplay reduced chemotaxis and lack of cell-polarization in response to MCP-1 and fMLP 55
LAT (Linker for activation of T cells): A novel immunohistochemical marker for T, NK, mast cells and megakaryocytes. Evaluation in normal and pathological conditions. 54
THE DEVELOPMENT OF FETAL IMMUNOCOMPETENCE - ANALYSIS OF LYMPHOID SUBSETS AND FUNCTION IN MID-TRIMESTER - APPLICATION TO PRENATAL DIAGNOSIS OF PRIMARY IMMUNODEFICIENCIES 53
Characterization of T and B cell repertoire diversity in patients with RAG deficiency 53
Immunohistologic analysis of ineffective CD40-CD40 ligand interaction in lymphoid tissues from patients with X-linked immunodeficiency with Hyper-IgM. 51
Totale 9.351
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Categoria #
all - tutte 55.986
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 55.986
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2019/20201.392 0 0 0 0 0 0 260 288 182 417 87 158
2020/20211.751 27 188 35 206 77 224 77 208 211 237 202 59
2021/20221.047 62 235 12 54 21 37 73 49 43 134 96 231
2022/2023887 147 24 24 56 96 219 1 101 138 4 38 39
2023/2024913 49 12 94 87 44 161 37 40 231 7 2 149
2024/2025683 7 9 3 279 228 157 0 0 0 0 0 0
Totale 10.443