IRIS Institutional Research Information System - OPENBS Open Archive UniBS

NOTARANGELO, Luigi Daniele
 Distribuzione geografica
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Continente #
NA - Nord America 6.043
EU - Europa 2.084
AS - Asia 1.250
OC - Oceania 10
AF - Africa 6
Continente sconosciuto - Info sul continente non disponibili 4
SA - Sud America 2
Totale 9.399
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Nazione #
US - Stati Uniti d'America 6.030
CN - Cina 888
UA - Ucraina 749
DE - Germania 393
HK - Hong Kong 222
FI - Finlandia 211
GB - Regno Unito 202
IT - Italia 154
IE - Irlanda 129
FR - Francia 73
IN - India 64
SE - Svezia 50
PL - Polonia 47
RU - Federazione Russa 29
TR - Turchia 23
SG - Singapore 21
BE - Belgio 18
NL - Olanda 16
CA - Canada 12
AU - Australia 10
IR - Iran 10
JP - Giappone 8
MU - Mauritius 5
EU - Europa 4
VN - Vietnam 4
BD - Bangladesh 2
EE - Estonia 2
GR - Grecia 2
KZ - Kazakistan 2
LU - Lussemburgo 2
TH - Thailandia 2
AL - Albania 1
AT - Austria 1
BA - Bosnia-Erzegovina 1
BR - Brasile 1
CH - Svizzera 1
IL - Israele 1
IS - Islanda 1
KR - Corea 1
MM - Myanmar 1
MX - Messico 1
PE - Perù 1
RO - Romania 1
SA - Arabia Saudita 1
SK - Slovacchia (Repubblica Slovacca) 1
SL - Sierra Leone 1
Totale 9.399
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Città #
Fairfield 950
Woodbridge 742
Houston 557
Jacksonville 542
Ashburn 447
Ann Arbor 418
Cambridge 395
Seattle 342
Chandler 306
Wilmington 293
Princeton 243
Hong Kong 222
Nanjing 205
Beijing 143
Dublin 129
Helsinki 117
New York 111
Nanchang 84
Des Moines 63
Changsha 58
Jinan 54
Lancaster 51
Shenyang 48
San Diego 47
Warsaw 47
Hebei 46
Brescia 45
Dearborn 42
Milan 32
London 29
Tianjin 29
Jiaxing 28
Zhengzhou 27
Shanghai 23
San Francisco 20
Brussels 18
Kunming 18
Hangzhou 17
Kocaeli 17
Verona 16
Ningbo 15
Lanzhou 14
Taizhou 14
Guangzhou 13
Haikou 13
Augusta 11
Boardman 10
Leawood 10
Norwalk 10
San Mateo 9
Taiyuan 9
Toronto 9
Ardabil 8
Washington 8
Changchun 7
Pune 7
Chiyoda-ku 6
Los Angeles 6
New Bedfont 6
Islington 5
Kilburn 5
Nürnberg 5
Acton 4
Chiswick 4
Dong Ket 4
Melbourne 4
Mumbai 4
Aubervilliers 3
Gunzenhausen 3
Orange 3
Prescot 3
Sydney 3
Almaty 2
Bangalore 2
Bangkok 2
Canberra 2
Cavriago 2
Chicago 2
Curtatone 2
Falkenstein 2
Florence 2
Fuzhou 2
Hefei 2
Hesperange 2
Istanbul 2
Munich 2
Parma 2
Philadelphia 2
Providence 2
Redmond 2
Redwood City 2
Sabz 2
Sheffield 2
Tallinn 2
Treviso 2
Urgnano 2
Walnut 2
Wandsworth 2
Amsterdam 1
Andover 1
Totale 7.310
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Nome #
Wiskott–Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma 160
Functional Characterization Of Natural Killer Cells In Type I Leukocyte Adhesion Deficiency 142
Lack of specific T- and B-cell clonal expansions in multiple sclerosis patients with progressive multifocal leukoencephalopathy 138
A novel activation-induced cytidine deaminase gene mutation in a Tunisian family with hyper IgM syndrome. 131
Monocytes from Wiskott-Aldrich patients differentiate in functional mature dendritic cells with a defect in CD83 expression 127
Pancreatitis in systemic lupus erythematosus. 127
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 126
X-linked agammaglobulinemia, growth hormone deficiency and delay of growth and puberty. 123
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 117
Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency 117
Signaling via IL-2 and IL-4 in JAK3-deficient severe combined immunodeficiency lymphocytes: JAK3-dependent and independent pathways. 116
Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome. 115
Stem cell transplantation for the Wiskott-Aldrich syndrome: a single-center experience confirms efficacy of matched unrelated donor transplantation 115
G-CSF treatment of Severe Congenital Neutropenia reverses neutropenia but does not correct the underlying functional deficiency of the neutrophil in defending against microorganisms. 114
Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience 114
Structural basis for SH2D1A mutations in X-linked lymphoproliferative disease. 113
In vitro cell death of activated lymphocytes in Omenn's syndrome 111
Western Blot technique in the serological evaluation of three LAV/HTLV III-infected Italian families. 110
AIRE deficiency in thymus of 2 patients with Omenn syndrome. 109
Cartilage-hair hypoplasia: molecular basis and heterogeneity of the immunological phenotype. 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
Cellular and molecular pathogenesis of X-linked lymphoproliferative disease. 106
Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment 105
Studies of the expression of the Wiskott-Aldrich syndrome protein. 103
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia 103
V(D)J recombination defects in lymphocytes due to RAG mutations: severe immunodeficiency with a spectrum of clinical presentations. 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
First report of successful stem cell transplantation in a child with CD40 deficiency 103
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
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 101
Altered leukocyte response to CXCL12 in patients with Warts Hypogammaglobulinemia, Infections, Myelokathexis (WHIM) syndrome 100
Natural Killer cell deficiencies and severe varicella infection 99
Activity of classical and alternative pathways of complement in preterm and small for gestational age infants. 99
Leukocyte adhesion deficiency in a child with severe oral involvement. 99
Long-term immune reconstitution and clinical outcome after stem cell transplantation for severe T-cell immunodeficiency 98
Mutations of the X-linked lymphoproliferative disease gene SH2D1A mimicking common variable immunodeficiency. 97
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
CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2-mediated condition 95
AIRE and immunological tolerance: insights from the study of autoimmune polyendocrinopathy candidiasis and ectodermal dystrophy 94
A combined immunodeficiency with severe infections, inflammation and allergy caused by ARPC1B deficiency 94
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
Reduced thymic output, increased spontaneous apoptosis and oligoclonal B cells in polyethylene glycol-adenosine deaminase-treated patients 92
F-BAR domain only protein 1 (FCHO1) deficiency is a novel cause of combined immune deficiency in human subjects 92
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations. 90
“Maturazione della risposta anticorpale: dal repertorio anticorpale primario a quello secondario”. 89
The spleen in the Wiskott-Aldrich syndrome: histopathologic abnormalities of the white pulp correlate with the clinical phenotype of the disease. 89
Defective migration of monocyte-derived dendritic cells in LAD-1 immunodeficiency. 89
Interleukin-15 (IL-15) induces IL-8 and monocyte chemotactic protein 1 production in human monocytes. 88
Omenn syndrome in an infant with IL7RA gene mutation 86
Defective expression of HLA class I and CD1a molecules in a boy with a Marfan-like phenotype and deep skin ulcers. 84
Isolation of Cosmid and cDNA Clones in the Region Surrounding the BTK Gene at Xq21.3-q22. 84
Cerebellar involvement in warts Hypogammaglobulinemia immunodeficiency myelokathexis patients: Neuroimaging and clinical findings 84
Jak3, severe combined immunodeficiency, and a new class of immunosuppressive drugs 83
Intrathymic restriction and peripheral expansion of the T cell repertoire in Omenn syndrome 83
Immunological and genetic bases of new primary immunodeficiencies 83
Impaired thymic output and restricted T-cell repertoire in two infants with immunodeficiency and early-onset generalized dermatitis 79
Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hot spots, effect on transcription and translation and phenotype/genotype correlation. 79
In vitro correction of JAK3-deficient severe combined immunodeficiency by retroviral-mediated gene transduction. 79
Wiskott-Aldrich syndrome. A model for defective actin reorganization, cell trafficking and synapse formation 78
Low WASp expression in patients with no apparent mutation in the WASP gene 76
WASP regulates suppressor activity of human and murine CD4+CD25+FOXP3+natural regulatory T cells 74
Expansion of large lymphocyte subsets in Wiskott-Aldrich syndrome. 73
WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function 72
IgG subclasses serum levels in juvenile chronic arthritis. 72
Cutaneous CD30+ cells in children with atopic dermatitis 72
Cooperative intervention to assist children with HIV-infection in Burkina Faso: The Brescia experience during the pilot phase of the ESTHER Project. 70
Long-term outcome and lineage-specific chimerism in 194 Wiskott-Aldrich Syndrome patients treated by hematopoietic celltransplantation between 1980-2009: an international collaborative study. 68
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 67
Expression of inducible Nitric Oxide Synthase (iNOS) in monocytic cells from children with infections by intracellular pathogens 67
IgM and IgG concentrations in the serum and secretions of children with selective IgA deficiency. 65
Serum IgG levels and complement activity in hypogammaglobulinemic patients under substitution therapy. 65
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 64
WASP-WIP DEFICIENCIES: NEW INSIGHTS IN WASP-WIP INTERACTION 63
CHARACTERIZATION OF AUTOANTIBODY PROFILE AMONG PATIENTS WITH PRIMARY IMMUNODEFICIENCY SECONDARY TO RAG MUTATION 63
Multi-lineage analysis of X-inactivation in female carriers of genetic alterations in the Wiskott-Aldrich syndrome protein (WASP) gene locus 62
Characterization of nine novel mutations in the CD40 ligand gene in patients with X-linked hiper IgM syndrome of various ancestry. 60
Ineffective expression of CD40L on cord blood T cells may contribute to poor immunoglobulin production in the newborn. 58
CD4+ cells from patients with common variable immunodeficiency are intrinsically defective in their ability to express membrane CD40 ligand after in vitro stimulation. 57
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 57
A new immunoperoxidase assay for lolium perenne-specific IgE in serum based on the biotin/avidin system (BAS). 56
Primary immune deficiencies unravel the molecular basis of immune response 55
Working Model of home Care for Families with HIV infectes children. 53
Lack of iNKT Cells and Defects on Differentiation of Dendritic Cells in Patients with Hermansky-Pudlak Type 2 Syndrome (HPS2) 51
SP110 REGULATES NUCLEAR ORPHAN RECEPTOR NUR77-DRIVEN APOPTOSIS IN T CELLS 51
Henoch-Schoenlein syndrome and selective IgA deficiency. 51
Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Diseases: Current Status and Future Perspectives. 51
LAT (Linker for activation of T cells): A novel immunohistochemical marker for T, NK, mast cells and megakaryocytes. Evaluation in normal and pathological conditions. 50
Auto-antibodies against type I IFNs in patients with life-threatening COVID-19 50
Monocytes from Wiskott-Aldrich patients desplay reduced chemotaxis and lack of cell-polarization in response to MCP-1 and fMLP 50
Major histocompatibility class II molecole deficiency (bare lymphocyte sindrome): Cell type specific residual expression of classical MHC protein on dendritic cells and endothelium. 49
Inborn Errors of Immunity With Immune Dysregulation: From Bench to Bedside. 49
Immunohistologic analysis of ineffective CD40-CD40 ligand interaction in lymphoid tissues from patients with X-linked immunodeficiency with Hyper-IgM. 48
THE DEVELOPMENT OF FETAL IMMUNOCOMPETENCE - ANALYSIS OF LYMPHOID SUBSETS AND FUNCTION IN MID-TRIMESTER - APPLICATION TO PRENATAL DIAGNOSIS OF PRIMARY IMMUNODEFICIENCIES 48
Mutations of CD40 gene cause a novel autosomal recessive form of hyper IgM (HIGM3). 47
Impaired natural and CD16-mediated NK cell cytotoxic function in WAS and XLT patients: ability of IL-2 to correct NK cell functional defect 47
Totale 8.683
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Categoria #
all - tutte 44.776
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 44.776
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2018/2019519 0 0 0 0 0 0 0 0 0 0 270 249
2019/20202.745 409 74 92 338 152 288 260 288 182 417 87 158
2020/20211.751 27 188 35 206 77 224 77 208 211 237 202 59
2021/20221.048 62 235 12 54 21 37 73 49 43 134 96 232
2022/2023894 148 24 24 56 98 220 1 102 140 4 38 39
2023/2024768 50 12 96 88 44 161 37 40 233 7 0 0
Totale 9.623