نتائج البحث - "WASp, Wiskott-Aldrich syndrome protein"

1

Lentiviral gene therapy corrects platelet phenotype and function in patients with Wiskott-Aldrich syndrome

المساهمون: Sereni, L., Castiello, M. C., Di Silvestre, D., Della Valle, P., Brombin, C., Ferrua, Paolo, Cicalese, M. P., Pozzi, L., Migliavacca, M., Bernardo, M. E., Pignata, C., Farah, R., Notarangelo, L. D., Marcus, N., Cattaneo, L., Spinelli, M., Giannelli, S., Bosticardo, M., van Rossem, K., D'Angelo, A., Aiuti, A., Mauri, P., Villa, A.

المصدر: Journal of allergy and clinical immunology 144 (2019): 825–838. doi:10.1016/j.jaci.2019.03.012
info:cnr-pdr/source/autori:Sereni L1, Castiello MC1, Di Silvestre D2, Della Valle P3, Brombin C4, Ferrua F5, Cicalese MP6, Pozzi L3, Migliavacca M6, Bernardo ME6, Pignata C7, Farah R8, Notarangelo LD9, Marcus N10, Cattaneo L11, Spinelli M12, Giannelli S1, Bosticardo M1, van Rossem K13, D'Angelo A3, Aiuti A5, Mauri P2, Villa A14./titolo:Lentiviral gene therapy corrects platelet phenotype and function in patients with Wiskott-Aldrich syndrome/doi:10.1016%2Fj.jaci.2019.03.012/rivista:Journal of allergy and clinical immunology/anno:2019/pagina_da:825/pagina_a:838/intervallo_pagine:825–838/volume:144
The Journal of Allergy and Clinical Immunology

مصطلحات موضوعية: 0301 basic medicine, Male, δ-g, Electron-dense granule, P-selectin, Wiskott–Aldrich syndrome, Genetic enhancement, sCD40L, Soluble CD40 ligand, vWF, von Willebrand factor, HSCT, Hematopoietic stem cell transplantation, LV, Lentivirus, 0302 clinical medicine, Immunology and Allergy, Medicine, MFI, Mean fluorescence intensity, Platelet, Child, platelet, biology, Wiskott-Aldrich syndrome, Hematopoietic Stem Cell Transplantation, X-linked thrombocytopenia, Phenotype, gene therapy, 3. Good health, 030220 oncology & carcinogenesis, Child, Preschool, platelets, Female, PRP, Platelet-rich plasma, Wiskott-Aldrich Syndrome Protein, Adult, Blood Platelets, BAFF, B cell–activating factor, Adolescent, Immunology, Article, ADP, Adenosine diphosphate, 03 medical and health sciences, CD62P, P-selectin, Von Willebrand factor, XLT, X-linked thrombocytopenia, Microscopy, Electron, Transmission, WASp, Wiskott-Aldrich syndrome protein, HMGB1, High-mobility group box 1, Humans, B-cell activating factor, GT, Gene therapy, business.industry, TEM, Transmission electron microscopy, Platelet Count, Lentivirus, FU, Follow-up, Infant, Genetic Therapy, medicine.disease, Platelet Activation, OCS, Open canalicular system, STAT3, Signal transducer and activator of transcription 3, 030104 developmental biology, CT, Closure time, GPX1, Glutathione peroxidase 1, Platelet-rich plasma, sCD62P, Soluble P-selectin, FERMT3, Fermitin family homolog 3, WAS, Wiskott-Aldrich syndrome, biology.protein, business, HD, Healthy donor, ROS, Reactive oxygen species

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8fbef2a3b9359481a3aee0ecf5da45bbTest
https://publications.cnr.it/doc/417797Test

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Platelet Rho GTPases–a focus on novel players, roles and relationships

المؤلفون: Goggs, Robert, Williams, Christopher M., Mellor, Harry, Poole, Alastair W.

المصدر: Biochemical Journal

مصطلحات موضوعية: rho GTP-Binding Proteins, Syk, spleen tyrosine kinase, Platelet Aggregation, TRIO, triple functional domain containing protein, Review Article, Cdc42, cell division control protein 42, Rac, Ras-related C3 botulinum toxin substrate, ROCK, Rho kinase, MLC, myosin light chain, shape change, Ena, enabled, MTSS1, metastasis suppressor protein 1, FcR, Fc receptor, AP-MS, affinity-purification mass spectrometry, ARP2/3, actin related protein 2/3 complex, Rif, Rho in filopodia (RhoF), WASP, Wiskott–Aldrich syndrome protein, ECM, extracellular matrix, LARG, leukaemia-associated Rho GEF (ARHGEF12), secretion, PI3K, phosphoinositide 3-kinase, PREX-1, PI(3,4,5)P3-dependent Rac exchanger 1 protein, Signal Transduction, VWF, von Willebrand factor, Blood Platelets, PAR, protease activated receptor, NSF, N-ethylmaleimide-sensitive factor, CRP, collagen-related peptide, SNARE, soluble N-ethylmaleimide attachment protein receptors, PF4, platelet factor-4, GEF, guanine nucleotide exchange factor, PLC, phospholipase C, PAK, P21-activated kinase, LAT, linker for activation of T-cells, PKC, protein kinase C, Animals, Humans, cell division control protein 42 (Cdc42), thrombosis, Rho GTPase, Ras homology family GTPase, Rif, ITAM, immunoreceptor tyrosine-based activation motif, Platelet Activation, GDI, guanine nucleotide dissociation inhibitor, RhoG, IMD, IRSp53 and MIM homology domain, ELMO, engulfment and cell motility, VAMP, vesicle-associated membrane protein, F-actin, filamentous actin, TCR, T-cell receptor, GAP, GTPase activating protein, ARHGEF, Rho guanine nucleotide exchange factor, GP, glycoprotein, VASP, vasodilator-stimulated phosphoprotein, DOCK, dedicator of cytokinesis

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=pmid________::8dee12c58f70730a3a55910e7d6987a6Test
http://europepmc.org/articles/PMC4357237Test

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Gene therapy for PIDs: Progress, pitfalls and prospects

المؤلفون: Sayandip Mukherjee, Adrian J. Thrasher

المصدر: Gene
Gene; Vol 525

مصطلحات موضوعية: NADPH, nicotinamide adenine dinucleotide phosphate hydrogen, Adenosine Deaminase, ADA-SCID, adenosine deaminase deficiency-severe combined immunodeficiency, XLN, X-linked neutropenia, Genetic enhancement, MN, meganucleases, Review, Bioinformatics, Granulomatous Disease, Chronic, X-Linked Combined Immunodeficiency Diseases, miR, microRNA, SIN, self-inactivating, 0302 clinical medicine, NK cells, natural killer cells, Agammaglobulinemia, RVs, retroviral vectors, Transgenes, HSCs, haematopoietic stem cells, 0303 health sciences, Clinical Trials as Topic, GALV, gibbon ape leukaemia virus, UCOE, ubiquitous chromatin opening element, Hematopoietic Stem Cell Transplantation, General Medicine, GSH, genomic safe harbour, 3. Good health, ERT, enzyme replacement therapy, LVs, lentiviral vectors, 030220 oncology & carcinogenesis, HSCT, MDS, myelodysplastic syndrome, SCID-X1, X-linked severe combined immunodeficiency, WAS, Transgene, DSB, double strand break, GvHD, graft versus host disease, CGD, chronic granulomatous disorder, PGK, phosphoglycerokinase, Biology, HSCT, haematopoietic stem cell transplant, SCID, Transplantation, Autologous, Viral vector, 03 medical and health sciences, ROS, reactive oxygen species, Gene therapy, WAS, Wiskott–Aldrich syndrome, XLT, X-linked thrombocytopenia, CGD, ZFN, zinc finger nuclease, medicine, Genetics, Humans, IFN, interferon, TALEN, transcription activator-like effector nucleases, T-ALL, T cell-acute lymphoblastic leukaemia, 030304 developmental biology, PEG, polyethylene glycol, Severe combined immunodeficiency, SAE, serious adverse event, HLA, human leukocyte antigen, VSV-G, vesicular stomatitis virus-G protein, WASp, Wiskott–Aldrich syndrome protein, PID, Immunologic Deficiency Syndromes, MLV, murine leukaemia virus, Genetic Therapy, medicine.disease, Hematopoietic Stem Cells, IL, interleukin, Clinical trial, Transplantation, Mutagenesis, Insertional, Immunology, Primary immunodeficiency, Severe Combined Immunodeficiency, PIDs, primary immunodeficiency disorders, LTR, long terminal repeat, EF-1α, elongation factor 1α, HR, homologous recombination, SFFV, spleen focus-forming virus, LCR, locus control region

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7d90bb8143387c882595f8cb2bb2b67eTest

4

Maintaining intestinal health: the genetics and immunology of very early onset inflammatory bowel disease

المؤلفون: David Artis, Robert N. Baldassano, Gregory F. Sonnenberg, Judith R. Kelsen

المصدر: Cellular and Molecular Gastroenterology and Hepatology
Cellular and Molecular Gastroenterology and Hepatology, Vol 1, Iss 5, Pp 462-476 (2015)

مصطلحات موضوعية: Disease, Review, Inflammatory bowel disease, Whole Exome Sequencing, Pathogenesis, 0302 clinical medicine, Genetics, XIAP, X-linked inhibitor of apoptosis protein, 0303 health sciences, education.field_of_study, TNF, tumor necrosis factor, IBD, inflammatory bowel disease, TTC7A, tetratricopeptide repeat domain-containing protein 7A, Innate lymphoid cell, Gastroenterology, IPEX, immunodysregulation, polyendocrinopathy, and enteropathy, X-linked, FOXP3, Mucosal Immunology, COL7A1, collagen, type VII, α1, MHCII, major histocompatibility complex class II, WASP, Wiskott-Aldrich syndrome protein, 3. Good health, STAT, signal transducer and activator of transcription, VEO-IBD, very early onset inflammatory bowel disease, 030211 gastroenterology & hepatology, CVID, common variable immunodeficiency, WES, whole exome sequencing, Population, NEMO, nuclear factor-κB essential modulator, Very Early Onset Inflammatory Bowel Disease, Treg, regulatory T cell, Biology, GWAS, genomewide association studies, ILC3, group 3 innate lymphoid cells, IgA, immunoglobulin A, digestive system, 03 medical and health sciences, medicine, RAG, recombination-activating gene, lcsh:RC799-869, education, 030304 developmental biology, ADAM17, A disintegrin and metalloproteinase domain 17, Hepatology, Common variable immunodeficiency, Inflammatory Bowel Disease, ILC, innate lymphoid cells, CGD, chronic granulomatous disease, IKBKG, inhibitor of κ light polypeptide gene enhancer in B cells, kinase of, γ, FOXP3, forkhead box protein 3, GUCY2, guanylate cyclase 2, medicine.disease, digestive system diseases, IL, interleukin, Mucosal immunology, Immunology, lcsh:Diseases of the digestive system. Gastroenterology

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::851656cf50a99b459dbfa8812ec6bd4bTest
https://pubmed.ncbi.nlm.nih.gov/26393237Test

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Experimental Models for Studying Food Allergy

المؤلفون: Edda Fiebiger, Cynthia Kanagaratham, Benjamin F. Sallis

المصدر: Cellular and Molecular Gastroenterology and Hepatology

مصطلحات موضوعية: 0301 basic medicine, Allergy, Allergen Sensitization, Review, Disease, Allergic sensitization, Mice, 0302 clinical medicine, Global health, Murine Models of Food Allergy, LCT, long chain triglycerides, Mice, Knockout, Mice, Inbred BALB C, Gastroenterology, OIT, oral immunotherapy, WASP, Wiskott–Aldrich syndrome protein, EPIT, epicutaneous immunotherapy, IgE, GM-CSF, granulocyte-macrophage colony-stimulating factor, Food Hypersensitivity, PBMC, peripheral blood mononuclear cell, medicine.medical_specialty, Treg, regulatory T cell, MCT, medium chain triglycerides, Receptors, Cell Surface, FCERIA, 03 medical and health sciences, Allergen Challenge, HSC, hematopoietic stem cell, Food allergy, Epictutaneous Sensitization, Intervention (counseling), medicine, Animals, Humans, TSLP, thymic stromal lymphopoietin, Intensive care medicine, Anaphylaxis, Th, T helper, Hepatology, Receptors, IgE, business.industry, Mechanism (biology), Allergens, Immunoglobulin E, medicine.disease, Spontaneous Sensitization, Ig, immunoglobulin, IL, interleukin, FCER1A, high-affinity immunoglobulin epsilon receptor subunit alpha, Disease Models, Animal, Humanized Model, 030104 developmental biology, Food, FcεRI, high-affinity immunoglobulin E receptor, MCPT, mouse mast cell protease, Immunization, business, Human Pathology, 030215 immunology

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6f78401abb53a7d06ecc788236dd87e0Test
https://doi.org/10.1016/j.jcmgh.2018.05.010Test

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Reduced type I interferon production by dendritic cells and weakened antiviral immunity in patients with Wiskott-Aldrich syndrome protein deficiency

المؤلفون: Stefanie Scheu, Tak W. Mak, Stephanie Borkens, Gerben Bouma, Dieter Häussinger, Philipp A. Lang, Nadine Honke, Adrian J. Thrasher, Namir Shaabani, Carmen Barthuber, Sarah Booth, Andreas Meryk, Mike Recher, Dirk Brenner, Gillian M. Griffiths, Karl S. Lang, Pamela S. Ohashi

المصدر: The Journal of Allergy and Clinical Immunology

مصطلحات موضوعية: pfu, Plaque-forming units, Medizin, pDC, Plasmacytoid dendritic cell, Plasmacytoid dendritic cell, CD8-Positive T-Lymphocytes, cDC, Conventional dendritic cell, Mice, 0302 clinical medicine, NK, Natural killer, Interferon, Lymphocytic choriomeningitis virus, Immunology and Allergy, Immunodeficiency, Mice, Knockout, Wiskott-Aldrich syndrome protein, 0303 health sciences, diabetes, biology, Wiskott-Aldrich syndrome, Wiskott–Aldrich syndrome protein, IL-7R, IL-7 receptor, WASP, Wiskott-Aldrich syndrome protein, DC, Dendritic cell, FACS, Fluorescence-activated cell sorting, LCMV, Lymphocytic choriomeningitis virus, 3. Good health, WAS KO, WASP knockout, Interferon Type I, TLR, Toll-like receptor, medicine.drug, Immunology, CD8 T cells, virus, macromolecular substances, Lymphocytic choriomeningitis, Vesicular stomatitis Indiana virus, 03 medical and health sciences, Immune system, Immune Deficiencies, Infection, and Systemic Immune Disorders, YFP, Yellow fluorescent protein, Rhabdoviridae Infections, medicine, Animals, Arenaviridae Infections, Humans, dendritic cells, Type I interferon, 030304 developmental biology, IFN-I, Type I interferon, Poly(I:C), Polyinosine-polycytidylic acid, medicine.disease, Type I interferon production, Virology, Mice, Inbred C57BL, Disease Models, Animal, WAS, Wiskott-Aldrich syndrome, VSV, Vesicular stomatitis virus, biology.protein, Interferon type I, 030215 immunology

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::97b836e174cf3cd01f2bfcc1dc29b222Test
https://www.ncbi.nlm.nih.gov/pubmed/23141740Test

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Dysbindin-Containing Complexes and their Proposed Functions in Brain: From Zero to (too) Many in a Decade

المؤلفون: Esteban C. Dell'Angelica, Cristina A. Ghiani

المصدر: ASN Neuro, Vol 3 (2011)
ASN NEURO

مصطلحات موضوعية: MS/MS, tandem mass spectrometry, Dendritic spine, Review Article, DTNBP1, Dystrophin, Mice, Transcriptional regulation, Mice, Knockout, Neurons, siRNA, small-interfering RNA, Behavior, Animal, General Neuroscience, Glutamate receptor, Brain, Dystrophin-associated protein, HEK-293 cells, human embryonic kidney cells, Cell biology, dystrobrevin-binding protein, Dysbindin, RNAi, RNA interference, coIP, co-immunoprecipitation, HPS, Hermansky–Pudlak syndrome, JNK, c-Jun N-terminal kinase, WASH, Wiskott–Aldrich syndrome protein and SCAR homologue, VAMP-7, vesicle-associated membrane protein 7, BLOC, biogenesis of lysosome-related organelles complex, WAVE, WASP (Wiskott–Aldrich syndrome protein) verprolin homologous, S1, dysbindin, Nerve Tissue Proteins, Biology, Synaptic vesicle, S4, lcsh:RC321-571, biogenesis of lysosome-related organelles complex-1 (BLOC-1), Animals, Humans, shRNA, short-hairpin RNA, lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry, AP-3, adaptor protein-3, Y2H, yeast two-hybrid, Wiskott-Aldrich Syndrome Protein Family, schizophrenia, Multiprotein Complexes, Dystrophin-Associated Proteins, Synapses, Neurology (clinical), Carrier Proteins, Neuroscience, Biogenesis, Function (biology)

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d1531a486ea853789d94aa833bd094e6Test
https://doaj.org/article/7e3e16b119ce445b847f8738f14a1297Test

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B-cell reconstitution after lentiviral vector-mediated gene therapy in patients with Wiskott-Aldrich syndrome

المؤلفون: Paolo Uva, Mirjam van der Burg, Anna Villa, Immacolata Brigida, Francesca Ferrua, Lucia Piceni Sereni, Michael H. Albert, Giorgio Ottaviano, Marita Bosticardo, Alessandro Aiuti, Maria Carmina Castiello, Francesca Pala, Maria Grazia Roncarolo, Luigi Naldini, Samantha Scaramuzza

المساهمون: Castiello, Mc, Scaramuzza, S, Pala, F, Ferrua, F, Uva, P, Brigida, I, Sereni, L, van der Burg, M, Ottaviano, G, Albert, Mh, Grazia Roncarolo, M, Naldini, Luigi, Aiuti, Alessandro, Villa, A, Bosticardo, M., Immunology

المصدر: Journal of Allergy and Clinical Immunology, 136(3), 692-+. Mosby Inc.
The Journal of Allergy and Clinical Immunology

مصطلحات موضوعية: Male, VCN, Vector copy number, Transplantation Conditioning, Wiskott–Aldrich syndrome, medicine.medical_treatment, Gene Expression, Hematopoietic stem cell transplantation, Bone Marrow, Transduction, Genetic, B-Cell Activating Factor, Immunology and Allergy, Child, BM, Bone marrow, B cell, Wiskott-Aldrich syndrome, Hematopoietic Stem Cell Transplantation, Hematopoietic stem cell, gene therapy, 3. Good health, medicine.anatomical_structure, Child, Preschool, SDF-1α, Stromal cell–derived factor 1α, Stem cell, Wiskott-Aldrich Syndrome Protein, BAFF, B cell–activating factor, Recombinant Fusion Proteins, Genetic Vectors, Immunology, B-Lymphocyte Subsets, Immunoglobulins, Biology, primary immunodeficiency, Transplantation, Autologous, CD19, Immunophenotyping, Immune Deficiencies, Infection, and Systemic Immune Disorders, WASp, Wiskott-Aldrich syndrome protein, medicine, Humans, Progenitor cell, Autoantibodies, IVIg, Intravenous immunoglobulin, GT, Gene therapy, Gene Expression Profiling, Lentivirus, lentiviral vector, Infant, PB, Peripheral blood, Genetic Therapy, HSC, Hematopoietic stem cell, Hematopoietic Stem Cells, medicine.disease, WAS, Wiskott-Aldrich syndrome, biology.protein, Bone marrow, HD, Healthy donor

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::495ffab946a30ddf28166ff0f448fa5aTest
https://doi.org/10.1016/j.jaci.2015.01.035Test

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رسالة جامعية

Investigation of the role of WIP and WASP in regulating myeloid cells migration during mild hyperthermia

المؤلفون: Ciccioli, Mariacristina

Committee Members: Calle-Patino, Yolanda; Letek Polberg, Michal

URL: https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.808622Test

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