Wiskott-Aldrich syndrome: a comprehensive review (Q33406620)

26 July 2017

title

Wiskott-Aldrich syndrome: a comprehensive review (English)

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26 July 2017

main subject

Wiskott-Aldrich syndrome

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3

Raif S Geha

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26 July 2017

Michel J Massaad

1

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26 July 2017

Narayanaswamy Ramesh

series ordinal

2

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26 July 2017

language of work or name

English

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publication date

25 March 2013

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Annals of the New York Academy of Sciences

26 July 2017

published in

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26 July 2017

volume

1285

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26 July 2017

page(s)

26-43

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Isolation of a novel gene mutated in Wiskott-Aldrich syndrome

26 July 2017

cites work

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The genotype of the original Wiskott phenotype

21 January 2018

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Disease mechanism: unravelling Wiskott-Aldrich syndrome

21 January 2018

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A multiinstitutional survey of the Wiskott-Aldrich syndrome.

21 January 2018

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X-linked thrombocytopenia and Wiskott-Aldrich syndrome are allelic diseases with mutations in the WASP gene

21 January 2018

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A large kindred with X-linked neutropenia with an I294T mutation of the Wiskott-Aldrich syndrome gene.

21 January 2018

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Constitutively activating mutation in WASP causes X-linked severe congenital neutropenia

21 January 2018

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The Wiskott-Aldrich syndrome protein (WASP): roles in signaling and cytoskeletal organization

21 January 2018

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Induction of filopodium formation by a WASP-related actin-depolymerizing protein N-WASP

21 January 2018

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21 January 2018

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Identification of two human WAVE/SCAR homologues as general actin regulatory molecules which associate with the Arp2/3 complex

21 January 2018

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Human subtelomeric WASH genes encode a new subclass of the WASP family

21 January 2018

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WHAMM is an Arp2/3 complex activator that binds microtubules and functions in ER to Golgi transport

21 January 2018

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p53-cofactor JMY is a multifunctional actin nucleation factor

21 January 2018

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Evolution of the eukaryotic ARP2/3 activators of the WASP family: WASP, WAVE, WASH, and WHAMM, and the proposed new family members WAWH and WAML

21 January 2018

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Studies of the expression of the Wiskott-Aldrich syndrome protein

21 January 2018

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WIP, a protein associated with Wiskott–Aldrich syndrome protein, induces actin polymerization and redistribution in lymphoid cells

21 January 2018

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WIP regulates the stability and localization of WASP to podosomes in migrating dendritic cells

21 January 2018

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WIP is a chaperone for Wiskott-Aldrich syndrome protein (WASP).

21 January 2018

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Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hotspots, effect on transcription, and translation and phenotype/genotype correlation

21 January 2018

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X-linked thrombocytopenia caused by a mutation in the Wiskott-Aldrich syndrome (WAS) gene that disrupts interaction with the WAS protein (WASP)-interacting protein (WIP)

21 January 2018

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Structure of the N-WASP EVH1 Domain-WIP Complex

21 January 2018

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Multiple WASP-interacting protein recognition motifs are required for a functional interaction with N-WASP

21 January 2018

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A peptide derived from the Wiskott-Aldrich syndrome (WAS) protein-interacting protein (WIP) restores WAS protein level and actin cytoskeleton reorganization in lymphocytes from patients with WAS mutations that disrupt WIP binding

21 January 2018

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WIP regulates N-WASP-mediated actin polymerization and filopodium formation

21 January 2018

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Toca-1 mediates Cdc42-dependent actin nucleation by activating the N-WASP-WIP complex

21 January 2018

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Requirement for a complex of Wiskott-Aldrich syndrome protein (WASP) with WASP interacting protein in podosome formation in macrophages

21 January 2018

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Wiskott-Aldrich syndrome protein is a key regulator of the phagocytic cup formation in macrophages

21 January 2018

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The Wiskott-Aldrich syndrome protein-interacting protein (WIP) binds to the adaptor protein Nck

21 January 2018

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Mechanism of recruitment of WASP to the immunological synapse and of its activation following TCR ligation

21 January 2018

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Autoinhibition and activation mechanisms of the Wiskott-Aldrich syndrome protein

21 January 2018

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The interaction between N-WASP and the Arp2/3 complex links Cdc42-dependent signals to actin assembly

21 January 2018

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Wiskott-Aldrich syndrome protein, a novel effector for the GTPase CDC42Hs, is implicated in actin polymerization

21 January 2018

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Activation by Cdc42 and PIP(2) of Wiskott-Aldrich syndrome protein (WASp) stimulates actin nucleation by Arp2/3 complex

21 January 2018

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Integration of multiple signals through cooperative regulation of the N-WASP-Arp2/3 complex

21 January 2018

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Mechanism of N-WASP activation by CDC42 and phosphatidylinositol 4, 5-bisphosphate

21 January 2018

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Phosphorylation of tyrosine 291 enhances the ability of WASp to stimulate actin polymerization and filopodium formation. Wiskott-Aldrich Syndrome protein

21 January 2018

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Contingent phosphorylation/dephosphorylation provides a mechanism of molecular memory in WASP.

21 January 2018

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Fyn and PTP-PEST-mediated regulation of Wiskott-Aldrich syndrome protein (WASp) tyrosine phosphorylation is required for coupling T cell antigen receptor engagement to WASp effector function and T cell activation

21 January 2018

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Sustained activation of N-WASP through phosphorylation is essential for neurite extension

21 January 2018

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Neuronal Wiskott-Aldrich syndrome protein (N-WASP) is critical for formation of α-smooth muscle actin filaments during myofibroblast differentiation

21 January 2018

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The Wiskott-Aldrich syndrome: The actin cytoskeleton and immune cell function

21 January 2018

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Identification of Itk/Tsk Src homology 3 domain ligands

21 January 2018

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Interaction between Wiskott-Aldrich Syndrome protein (WASP) and the Fyn protein-tyrosine kinase

21 January 2018

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Identification of Fyn as the binding partner for the WASP N-terminal domain in T cells

21 January 2018

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Wiskott-Aldrich syndrome protein physically associates with Nck through Src homology 3 domains

21 January 2018

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Wiskott-Aldrich syndrome protein is associated with the adapter protein Grb2 and the epidermal growth factor receptor in living cells

21 January 2018

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Nck and phosphatidylinositol 4,5-bisphosphate synergistically activate actin polymerization through the N-WASP-Arp2/3 pathway

21 January 2018

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GRB2 links signaling to actin assembly by enhancing interaction of neural Wiskott-Aldrich syndrome protein (N-WASp) with actin-related protein (ARP2/3) complex

21 January 2018

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Phosphatidylinositol 4,5-biphosphate (PIP2)-induced vesicle movement depends on N-WASP and involves Nck, WIP, and Grb2

21 January 2018

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The Wiskott-Aldrich syndrome protein acts downstream of CD2 and the CD2AP and PSTPIP1 adaptors to promote formation of the immunological synapse

21 January 2018

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Regulation of actin filament network formation through ARP2/3 complex: activation by a diverse array of proteins.

21 January 2018

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Influence of the C terminus of Wiskott-Aldrich syndrome protein (WASp) and the Arp2/3 complex on actin polymerization

21 January 2018

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Scar1 and the related Wiskott-Aldrich syndrome protein, WASP, regulate the actin cytoskeleton through the Arp2/3 complex

21 January 2018

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Interaction of WASP/Scar proteins with actin and vertebrate Arp2/3 complex

21 January 2018

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WASP localizes to the membrane skeleton of platelets

21 January 2018

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Phosphorylation of WASp is a key regulator of activity and stability in vivo

21 January 2018

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WASP and N-WASP in human platelets differ in sensitivity to protease calpain

21 January 2018

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Ubiquitylation-Dependent Negative Regulation of WASp Is Essential for Actin Cytoskeleton Dynamics

21 January 2018

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Early deficit of lymphocytes in Wiskott-Aldrich syndrome: possible role of WASP in human lymphocyte maturation

21 January 2018

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T cell lines characterize events in the pathogenesis of the Wiskott-Aldrich syndrome

21 January 2018

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Wiskott-Aldrich syndrome protein regulates lipid raft dynamics during immunological synapse formation.

21 January 2018

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Defective Th1 cytokine gene transcription in CD4 and CD8 T cells from Wiskott-Aldrich syndrome patients.

21 January 2018

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The Wiskott-Aldrich syndrome protein regulates CTL cytotoxicity and is required for efficient killing of B cell lymphoma targets.

21 January 2018

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Wiskott-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation

21 January 2018

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Antigen receptor-induced activation and cytoskeletal rearrangement are impaired in Wiskott-Aldrich syndrome protein-deficient lymphocytes

21 January 2018

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WIP is critical for T cell responsiveness to IL-2

21 January 2018

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A role for Wiskott-Aldrich syndrome protein in T-cell receptor-mediated transcriptional activation independent of actin polymerization

21 January 2018

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Differential roles for Wiskott-Aldrich syndrome protein in immune synapse formation and IL-2 production

21 January 2018

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The immunological synapse: a molecular machine controlling T cell activation

21 January 2018

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Three-dimensional segregation of supramolecular activation clusters in T cells

21 January 2018

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Functional rafts in cell membranes

21 January 2018

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Dynamic molecular interactions linking the T cell antigen receptor to the actin cytoskeleton.

21 January 2018

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The Wiskott-Aldrich syndrome protein permits assembly of a focused immunological synapse enabling sustained T-cell receptor signaling.

21 January 2018

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The interaction between Cdc42 and WASP is required for SDF-1-induced T-lymphocyte chemotaxis.

21 January 2018

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Activation of Wiskott-Aldrich syndrome protein and its association with other proteins by stromal cell-derived factor-1alpha is associated with cell migration in a T-lymphocyte line

21 January 2018

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WIP and WASP play complementary roles in T cell homing and chemotaxis to SDF-1alpha.

21 January 2018

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WASP deficiency leads to global defects of directed leukocyte migration in vitro and in vivo

21 January 2018

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Autoimmunity in Wiskott-Aldrich syndrome: risk factors, clinical features, and outcome in a single-center cohort of 55 patients

21 January 2018

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Autoimmunity in Wiskott-Aldrich syndrome

21 January 2018

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Naturally arising Foxp3-expressing CD25 CD4 regulatory T cells in immunological tolerance to self and non-self

21 January 2018

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Impaired in vitro regulatory T cell function associated with Wiskott-Aldrich syndrome

21 January 2018

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WASP regulates suppressor activity of human and murine CD4( )CD25( )FOXP3( ) natural regulatory T cells

21 January 2018

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Wiskott-Aldrich syndrome protein is required for regulatory T cell homeostasis

21 January 2018

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The Wiskott-Aldrich syndrome protein is required for the function of CD4( )CD25( )Foxp3( ) regulatory T cells

21 January 2018

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Defective inhibition of B-cell proliferation by Wiskott-Aldrich syndrome protein-deficient regulatory T cells

21 January 2018

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Development of IgA nephropathy-like glomerulonephritis associated with Wiskott–Aldrich syndrome protein deficiency

21 January 2018

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Phenotypic perturbation of B cells in the Wiskott-Aldrich syndrome.

21 January 2018

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B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice.

21 January 2018

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WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function

21 January 2018

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Wiskott-Aldrich syndrome protein (WASP) and N-WASP are critical for peripheral B-cell development and function

21 January 2018

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Impaired cell adhesion, apoptosis, and signaling in WASP gene-disrupted Nalm-6 pre-B cells and recovery of cell adhesion using a transducible form of WASp.

21 January 2018

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Evidence for defective transmembrane signaling in B cells from patients with Wiskott-Aldrich syndrome

21 January 2018

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WASp-deficient B cells play a critical, cell-intrinsic role in triggering autoimmunity

21 January 2018

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Clinical course of patients with WASP gene mutations

21 January 2018

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A role for PKCtheta in outside-in alpha(IIb)beta3 signaling.

21 January 2018

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Wiskott-Aldrich syndrome: qualitative platelet defects and short platelet survival

21 January 2018

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Flow cytometric analysis of platelets from childrenwith the Wiskott‐Aldrich syndrome reveals defects in platelet development, activation and structure

21 January 2018

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Platelet-associated IgAs and impaired GPVI responses in platelets lacking WIP.

21 January 2018

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Intravenous immunoglobulin, splenectomy, and antibiotic prophylaxis in Wiskott-Aldrich syndrome

21 January 2018

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Splenectomy in the management of the thrombocytopenia of the Wiskott-Aldrich syndrome

21 January 2018

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Deficiency in the Wiskott-Aldrich protein induces premature proplatelet formation and platelet production in the bone marrow compartment

21 January 2018

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Platelet survival in Wiskott-Aldrich syndrome

21 January 2018

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Rapid platelet turnover in WASP(-) mice correlates with increased ex vivo phagocytosis of opsonized WASP(-) platelets

21 January 2018

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Antiplatelet antibodies in WASP(-) mice correlate with evidence of increased in vivo platelet consumption

21 January 2018

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Impaired natural and CD16-mediated NK cell cytotoxicity in patients with WAS and XLT: ability of IL-2 to correct NK cell functional defect

21 January 2018

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Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses

21 January 2018

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The mature activating natural killer cell immunologic synapse is formed in distinct stages

21 January 2018

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The Wiskott-Aldrich syndrome protein regulates nuclear translocation of NFAT2 and NF-kappa B (RelA) independently of its role in filamentous actin polymerization and actin cytoskeletal rearrangement.

21 January 2018

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Cdc42-interacting protein-4 functionally links actin and microtubule networks at the cytolytic NK cell immunological synapse.

21 January 2018

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Formation of a WIP-, WASp-, actin-, and myosin IIA-containing multiprotein complex in activated NK cells and its alteration by KIR inhibitory signaling

21 January 2018

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WIP is essential for lytic granule polarization and NK cell cytotoxicity

21 January 2018

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IL-2 induces a WAVE2-dependent pathway for actin reorganization that enables WASp-independent human NK cell function.

21 January 2018

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Impaired NK-cell migration in WAS/XLT patients: role of Cdc42/WASp pathway in the control of chemokine-induced beta2 integrin high-affinity state

21 January 2018

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The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function.

21 January 2018

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Wiskott-Aldrich syndrome protein is required for homeostasis and function of invariant NKT cells

21 January 2018

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Configuration of human dendritic cell cytoskeleton by Rho GTPases, the WAS protein, and differentiation

21 January 2018

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Impaired dendritic-cell homing in vivo in the absence of Wiskott-Aldrich syndrome protein

21 January 2018

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Expression of Wiskott-Aldrich syndrome protein in dendritic cells regulates synapse formation and activation of naive CD8 T cells

21 January 2018

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Efficient antigen presentation of soluble, but not particulate, antigen in the absence of Wiskott-Aldrich syndrome protein.

21 January 2018

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NK cell activation by dendritic cells (DCs) requires the formation of a synapse leading to IL-12 polarization in DCs.

21 January 2018

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Chemotaxis of macrophages is abolished in the Wiskott-Aldrich syndrome

21 January 2018

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Wiskott-Aldrich syndrome protein regulates podosomes in primary human macrophages

21 January 2018

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The polarization defect of Wiskott-Aldrich syndrome macrophages is linked to dislocalization of the Arp2/3 complex.

21 January 2018

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The chemotactic defect in wiskott-Aldrich syndrome macrophages is due to the reduced persistence of directional protrusions

21 January 2018

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Cutting edge: the Wiskott-Aldrich syndrome protein is required for efficient phagocytosis of apoptotic cells

21 January 2018

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Impaired integrin-dependent function in Wiskott-Aldrich syndrome protein-deficient murine and human neutrophils

21 January 2018

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Clinical spectrum, pathophysiology and treatment of the Wiskott–Aldrich syndrome

21 January 2018

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X-linked thrombocytopenia (XLT) due to WAS mutations: clinical characteristics, long-term outcome, and treatment options

21 January 2018

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Genotype-proteotype linkage in the Wiskott-Aldrich syndrome

21 January 2018

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Wiskott-Aldrich syndrome. An immunologic deficiency disease involving the afferent limb of immunity

21 January 2018

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New insights into atopic dermatitis

21 January 2018

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Primary immune deficiencies with aberrant IgE production

21 January 2018

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Unregulated actin polymerization by WASp causes defects of mitosis and cytokinesis in X-linked neutropenia

21 January 2018

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Activating WASP mutations associated with X-linked neutropenia result in enhanced actin polymerization, altered cytoskeletal responses, and genomic instability in lymphocytes

21 January 2018

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Bone marrow transplantation in 26 patients with Wiskott-Aldrich syndrome from a single center

21 January 2018

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Complete Correction of the Wiskott-Aldrich Syndrome by Allogeneic Bone-Marrow Transplantation

21 January 2018

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Hematopoietic cell transplantation for Wiskott-Aldrich syndrome: advances in biology and future directions for treatment

21 January 2018

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Long-term outcome and lineage-specific chimerism in 194 patients with Wiskott-Aldrich syndrome treated by hematopoietic cell transplantation in the period 1980-2009: an international collaborative study

21 January 2018

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Stem-cell gene therapy for the Wiskott-Aldrich syndrome

21 January 2018

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Gene therapy for the Wiskott-Aldrich syndrome

21 January 2018

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Development of novel efficient SIN vectors with improved safety features for Wiskott-Aldrich syndrome stem cell based gene therapy

21 January 2018

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High-definition mapping of retroviral integration sites identifies active regulatory elements in human multipotent hematopoietic progenitors.

21 January 2018

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Insertional transformation of hematopoietic cells by self-inactivating lentiviral and gammaretroviral vectors

21 January 2018

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Physiological promoters reduce the genotoxic risk of integrating gene vectors

21 January 2018

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Lentiviral vector-mediated gene transfer in T cells from Wiskott-Aldrich syndrome patients leads to functional correction.

21 January 2018

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Large-scale manufacture and characterization of a lentiviral vector produced for clinical ex vivo gene therapy application

21 January 2018

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Diverse genomic integration of a lentiviral vector developed for the treatment of Wiskott-Aldrich syndrome

21 January 2018

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Lentiviral vectors transcriptionally targeted to hematopoietic cells by WASP gene proximal promoter sequences.

21 January 2018

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Lentiviral vectors targeting WASp expression to hematopoietic cells, efficiently transduce and correct cells from WAS patients.

21 January 2018

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Preclinical safety and efficacy of human CD34( ) cells transduced with lentiviral vector for the treatment of Wiskott-Aldrich syndrome

21 January 2018

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Improvement of migratory defects in a murine model of Wiskott-Aldrich syndrome gene therapy

21 January 2018

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Efficacy of gene therapy for Wiskott-Aldrich syndrome using a WAS promoter/cDNA-containing lentiviral vector and nonlethal irradiation.

21 January 2018

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Evidence for long-term efficacy and safety of gene therapy for Wiskott-Aldrich syndrome in preclinical models

21 January 2018

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Lentiviral-mediated gene therapy leads to improvement of B-cell functionality in a murine model of Wiskott-Aldrich syndrome

21 January 2018

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Dendritic cell functional improvement in a preclinical model of lentiviral-mediated gene therapy for Wiskott-Aldrich syndrome

21 January 2018

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Ubiquitous high-level gene expression in hematopoietic lineages provides effective lentiviral gene therapy of murine Wiskott-Aldrich syndrome

21 January 2018

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Wiskott-Aldrich syndrome protein deficiency leads to reduced B-cell adhesion, migration, and homing, and a delayed humoral immune response

21 January 2018

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Opposing Effects of PKCθ and WASp on Symmetry Breaking and Relocation of the Immunological Synapse

21 January 2018

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Long-term survival and transplantation of haemopoietic stem cells for immunodeficiencies: report of the European experience 1968–99

21 January 2018

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Long-term outcome following hematopoietic stem-cell transplantation in Wiskott-Aldrich syndrome: collaborative study of the European Society for Immunodeficiencies and European Group for Blood and Marrow Transplantation

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/NYAS.12049

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release_jqs6g54ldbhkngikuv2hm6gn6e

26 July 2017

Fatcat ID

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mapped directly with Wikidata item

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