THE WISKOTT-ALDRICH SYNDROME PROTEIN (WASP): Roles in Signaling and Cytoskeletal Organization

Rho GTPases and the Actin Cytoskeleton

1998 • 6,129 citations

Rho, Rac, and Cdc42 GTPases regulate the assembly of multimolecular focal complexes associated with actin stress fibers, lamellipodia, and filopodia

1995 • 4,387 citations

Protein modules and signalling networks

1995 • 2,447 citations

Three-dimensional segregation of supramolecular activation clusters in T cells

1998 • 2,316 citations

Serial triggering of many T-cell receptors by a few peptide–MHC complexes

1995 • 1,143 citations

Redistribution and Pinocytosis of Lymphocyte Surface Immunoglobulin Molecules Induced by Anti-Immunoglobulin Antibody

1971 • 1,093 citations

Isolation of a novel gene mutated in Wiskott-Aldrich syndrome

1994 • 1,053 citations

On the Crawling of Animal Cells

1993 • 1,020 citations

Structural basis for the binding of proline-rich peptides to SH3 domains

1994 • 1,016 citations

Wiskott–Aldrich Syndrome Protein, a Novel Effector for the GTPase CDC42Hs, Is Implicated in Actin Polymerization

1996 • 873 citations

Sequence motifs for calmodulin recognition

1997 • 871 citations

A Novel Adaptor Protein Orchestrates Receptor Patterning and Cytoskeletal Polarity in T-Cell Contacts

1998 • 708 citations

Human p21-activated kinase (Pak1) regulates actin organization in mammalian cells

1997 • 687 citations

N-WASP, a novel actin-depolymerizing protein, regulates the cortical cytoskeletal rearrangement in a PIP2-dependent manner downstream of tyrosine kinases.

1996 • 667 citations

Thrombin receptor ligation and activated rac uncap actin filament barbed ends through phosphoinositide synthesis in permeabilized human platelets

1995 • 665 citations

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

1998 • 656 citations

A Conserved Binding Motif Defines Numerous Candidate Target Proteins for Both Cdc42 and Rac GTPases

1995 • 625 citations

A multiinstitutional survey of the Wiskott-Aldrich syndrome

1994 • 612 citations

Rac and Cdc42 Induce Actin Polymerization and G1 Cell Cycle Progression Independently of p65PAK and the JNK/SAPK MAP Kinase Cascade

1996 • 571 citations

The Soluble Catalytic Domain of Membrane Type 1 Matrix Metalloproteinase Cleaves the Propeptide of Progelatinase A and Initiates Autoproteolytic Activation

1996 • 564 citations

Selective modulation of protein kinase C-Θ during T-cell activation

1997 • 558 citations

How profilin promotes actin filament assembly in the presence of thymosin β4

1993 • 532 citations

Sustained signaling leading to T cell activation results from prolonged T cell receptor occupancy. Role of T cell actin cytoskeleton.

1995 • 528 citations

Regulation of vinculin binding to talin and actin by phosphatidyl-inositol-4-5-bisphosphate

1996 • 522 citations

PEDIGREE DEMONSTRATING A SEX-LINKED RECESSIVE CONDITION CHARACTERIZED BY DRAINING EARS, ECZEMATOID DERMATITIS AND BLOODY DIARRHEA

1954 • 512 citations

Wiskott-Aldrich Syndrome Protein-Deficient Mice Reveal a Role for WASP in T but Not B Cell Activation

1998 • 494 citations

Wiskott-Aldrich syndrome

1968 • 422 citations

Vav is a regulator of cytoskeletal reorganization mediated by the T-cell receptor

1998 • 420 citations

Aeronautics before 1919

1971 • 413 citations

Defective antigen receptor-mediated proliferation of B and T cells in the absence of Vav

1995 • 411 citations

Defects in actin-cap formation in Vav-deficient mice implicate an actin requirement for lymphocyte signal transduction

1998 • 403 citations

Defective signalling through the T- and B-cell antigen receptors in lymphoid cells lacking the vav proto-oncogene

1995 • 401 citations

Making the T Cell Receptor Go the Distance: A Topological View of T Cell Activation

1997 • 399 citations

Two GTPases, Cdc42 and Rac, bind directly to a protein implicated in the immunodeficiency disorder Wiskott–Aldrich syndrome

1996 • 397 citations

Regulation of the polarization of T cells toward antigen-presenting cells by Ras-related GTPase CDC42.

1995 • 386 citations

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

1997 • 376 citations

The Wiskott-Aldrich syndrome: studies of lymphocytes, granulocytes, and platelets

1980 • 327 citations

Specific and redundant roles of Src and Fyn in organizing the cytoskeleton

1995 • 325 citations

Wiskott-Aldrich Syndrome Protein Physically Associates with Nck through Src Homology 3 Domains

1995 • 315 citations

Cofilin, a protein in porcine brain that binds to actin filaments and inhibits their interactions with myosin and tropomyosin

1984 • 312 citations

Defective T-cell receptor signalling and positive selection of Vav-deficient CD4+CDS+thymocytes

1995 • 300 citations

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

1995 • 284 citations

Spatial relationships of microtubule-organizing centers and the contact area of cytotoxic T lymphocytes and target cells.

1982 • 283 citations

The adaptor protein Nck links receptor tyrosine kinases with the serine-threonine kinase Pak1

1996 • 268 citations

SCAR, a WASP-related Protein, Isolated as a Suppressor of Receptor Defects in Late Dictyostelium Development

1998 • 268 citations

Cloning of a novel T-cell protein FYB that binds FYN and SH2-domain-containing leukocyte protein 76 and modulates interleukin 2 production

1997 • 265 citations

RAC Regulation of Actin Polymerization and Proliferation by a Pathway Distinct from Jun Kinase

1996 • 264 citations

Activation of Pak by membrane localization mediated by an SH3 domain from the adaptor protein Nck

1997 • 259 citations

NIK is a new Ste20-related kinase that binds NCK and MEKK1 and activates the SAPK/JNK cascade via a conserved regulatory domain

1997 • 257 citations

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

1978 • 256 citations

Wiskott-Aldrich Syndrome/X-Linked Thrombocytopenia: WASP Gene Mutations, Protein Expression, and Phenotype

1997 • 252 citations

Bee1, a Yeast Protein with Homology to Wiscott-Aldrich Syndrome Protein, Is Critical for the Assembly of Cortical Actin Cytoskeleton

1997 • 252 citations

Drosophila Photoreceptor Axon Guidance and Targeting Requires the Dreadlocks SH2/SH3 Adapter Protein

1996 • 248 citations

Cytoskeletal Polarization of T Cells Is Regulated by an Immunoreceptor Tyrosine-based Activation Motif–dependent Mechanism

1998 • 247 citations

The specific direct interaction of helper T cells and antigen-presenting B cells.

1986 • 246 citations

Chemotaxis of macrophages is abolished in the Wiskott‐Aldrich syndrome

1998 • 241 citations

Direct interaction of the Wiskott-Aldrich syndrome protein with the GTPase Cdc42.

1996 • 229 citations

The Wiskott-Aldrich syndrome and X-linked congenital thrombocytopenia are caused by mutations of the same gene

1995 • 211 citations

Nck, a melanoma cDNA encoding a cytoplasmic protein consisting of the src homology units SH2 and SH3

1990 • 209 citations

Faciogenital dysplasia protein (FGD1) and Vav, two related proteins required for normal embryonic development, are upstream regulators of Rho GTPases

1996 • 209 citations

Defective Actin Reorganization and Polymerization of Wiskott-Aldrich T Cells in Response to CD3-Mediated Stimulation

1997 • 206 citations

Characterization of a human lymphocyte surface sialoglycoprotein that is defective in Wiskott-Aldrich syndrome.

1984 • 203 citations

THE WISKOTT-ALDRICH SYNDROME A DISORDER WITH A POSSIBLE DEFECT IN ANTIGEN PROCESSING OR RECOGNITION

1968 • 197 citations

T cells of patients with the Wiskott-Aldrich syndrome have a restricted defect in proliferative responses.

1993 • 197 citations

Identification of Itk/Tsk Src Homology 3 Domain Ligands

1996 • 193 citations

Inhibition and reversal of capping by cytochalasin B, yinblastine and colchicine

1974 • 188 citations

Wiskott—Aldrich Syndrome: Qualitative Platelet Defects and Short Platelet Survival

1969 • 180 citations

Src-homology 3 domain of protein kinase p59fyn mediates binding to phosphatidylinositol 3-kinase in T cells.

1993 • 178 citations

PSTPIP: A Tyrosine Phosphorylated Cleavage Furrow–associated Protein that Is a Substrate for a PEST Tyrosine Phosphatase

1997 • 169 citations

Small splenic B cells that bind to antigen-specific T helper (Th) cells and face the site of cytokine production in the Th cells selectively proliferate: immunofluorescence microscopic studies of Th-B antigen-presenting cell interactions.

1994 • 168 citations

p56Lck and p59Fyn regulate CD28 binding to phosphatidylinositol 3-kinase, growth factor receptor-bound protein GRB-2, and T cell-specific protein-tyrosine kinase ITK: implications for T-cell costimulation.

1995 • 164 citations

Tyrosine-phosphorylated T cell receptor ζ chain associates with the actin cytoskeleton upon Activation of mature T lymphocytes

1995 • 162 citations

Wiskott–Aldrich syndrome protein (WASp) is a binding partner for c-Src family protein-tyrosine kinases

1996 • 154 citations

WASP gene mutations in Wiskott-Aldrich syndrome and X-linked thrombocytopenia

1995 • 151 citations

Concentration of an integral membrane protein, CD43 (leukosialin, sialophorin), in the cleavage furrow through the interaction of its cytoplasmic domain with actin-based cytoskeletons.

1993 • 148 citations

Splenectomy in the Management of the Thrombocytopenia of the Wiskott–Aldrich Syndrome

1980 • 145 citations

Monocytes from Wiskott-Aldrich patients display reduced chemotaxis and lack of cell polarization in response to monocyte chemoattractant protein-1 and formyl-methionyl-leucyl-phenylalanine.

1998 • 144 citations

Morphological abnormalities in the lymphocytes of patients with the Wiskott-Aldrich syndrome

1986 • 143 citations

The Cdc42/Rac Interactive Binding Region Motif of the Wiskott Aldrich Syndrome Protein (WASP) Is Necessary but Not Sufficient for Tight Binding to Cdc42 and Structure Formation

1998 • 143 citations

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

1992 • 141 citations

Direct Binding of the Verprolin-Homology Domain in N-WASP to Actin Is Essential for Cytoskeletal Reorganization

1998 • 141 citations

Isolation of a NCK-associated Kinase, PRK2, an SH3-binding Protein and Potential Effector of Rho Protein Signaling

1996 • 141 citations

The Wiskott-Aldrich Syndrome Protein-interacting Protein (WIP) Binds to the Adaptor Protein Nck

1998 • 139 citations

Characterization of cDNAs encoding human leukosialin and localization of the leukosialin gene to chromosome 16.

1989 • 138 citations

Tyrosine Phosphorylation Regulates the SH3-mediated Binding of the Wiskott-Aldrich Syndrome Protein to PSTPIP, a Cytoskeletal-associated Protein

1998 • 137 citations

Nonrandom inactivation of the X chromosome in early lineage hematopoietic cells in carriers of Wiskott-Aldrich syndrome

1995 • 137 citations

Molecular characterization of sialophorin (CD43), the lymphocyte surface sialoglycoprotein defective in Wiskott-Aldrich syndrome.

1989 • 135 citations

Monocytes from Wiskott-Aldrich Patients Display Reduced Chemotaxis and Lack of Cell Polarization in Response to Monocyte Chemoattractant Protein-1 and Formyl-Methionyl-Leucyl-Phenylalanine

1998 • 134 citations

Antigen-receptor complex stimulation triggers protein kinase C-dependent CD11a/CD18-cytoskeleton association in T lymphocytes.

1992 • 131 citations

SURFACE PROTEIN ABNORMALITIES IN LYMPHOCYTES AND PLATELETS FROM PATIENTS WITH WISKOTT-ALDRICH SYNDROME

1981 • 131 citations

Studies of the expression of the Wiskott-Aldrich syndrome protein.

1996 • 131 citations

Association of Ash/Grb-2 with dynamin through the Src homology 3 domain.

1994 • 125 citations

Defects in Wiskott-Aldrich syndrome blood cells

1996 • 122 citations

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

1997 • 120 citations

Actin-binding Verprolin Is a Polarity Development Protein Required for the Morphogenesis and Function of the Yeast Actin Cytoskeleton

1997 • 116 citations

fyn tyrosine kinase is involved in keratinocyte differentiation control.

1995 • 113 citations

Carrier detection in the Wiskott Aldrich syndrome

1988 • 113 citations

Actin polymerization and pseudopod reorganization accompany anti-CD3-induced growth arrest in Jurkat T cells.

1993 • 112 citations

Evidence that the Wiskott-Aldrich syndrome protein may be involved in lymphoid cell signaling pathways.

1996 • 109 citations

Ash/Grb-2, a SH2/SH3-containing protein, couples to signaling for mitogenesis and cytoskeletal reorganization by EGF and PDGF.

1993 • 108 citations

X-Linked Wiskott–Aldrich Syndrome in a Girl

1998 • 108 citations

Identification of WASP mutations in patients with Wiskott-Aldrich syndrome and isolated thrombocytopenia reveals allelic heterogeneity at the WAS locus

1995 • 105 citations

Identification of mutations in the Wiskott-Aldrich syndrome gene and characterization of a polymorphic dinucleotide repeat at DXS6940, adjacent to the disease gene.

1995 • 104 citations

Interaction between Sam68 and Src Family Tyrosine Kinases, Fyn and Lck, in T Cell Receptor Signaling

1997 • 103 citations

Molecular Cloning of SKAP55, a Novel Protein That Associates with the Protein Tyrosine Kinase p59 in Human T-lymphocytes

1997 • 102 citations

Syk Tyrosine Kinase Is Required for Immunoreceptor Tyrosine Activation Motif-dependent Actin Assembly

1996 • 100 citations

Cell-surface-expressed T-cell antigen-receptor zeta chain is associated with the cytoskeleton.

1995 • 98 citations

Wiskott-Aldrich syndrome: cellular impairments and their implication for carrier detection

1980 • 96 citations

Abnormalities of Chemotactic Lymphokine Synthesis and Mononuclear Leukocyte Chemotaxis in Wiskott-Aldrich Syndrome

1974 • 95 citations

ALLELIC EXCLUSION OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE IN PLATELETS AND T LYMPHOCYTES FROM A WISKOTT-ALDRICH SYNDROME CARRIER

1980 • 95 citations

A proline‐rich protein, verprolin, involved in cytoskeletal organization and cellular growth in the yeast Saccharomyces cerevisiae

1993 • 95 citations

X-Chromosome inactivation in the Wiskott-Aldrich syndrome: A marker for detection of the carrier state and identfication of cell lineages expressing the gene defect

1989 • 94 citations

Expression of Wiskott-Aldrich Syndrome Protein (WASP) Gene During Hematopoietic Differentiation

1997 • 92 citations

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

1992 • 91 citations

WASPbase: a database of WAS- and XLT-causing mutations

1996 • 89 citations

Identification of homer as a homologue of the Wiskott-Aldrich Syndrome protein suggests a receptor-binding function for WH1 domains

1997 • 89 citations

Wiskott-Aldrich syndrome: no strict genotype-phenotype correlations but clustering of missense mutations in the amino-terminal part of the WASP gene product

1996 • 88 citations

Association of various T cell-surface molecules with the cytoskeleton. Effect of cross-linking and activation.

1991 • 85 citations

Collagen Induces Tyrosine Phosphorylation of Wiskott-Aldrich Syndrome Protein in Human Platelets

1998 • 84 citations

Networking Rho Family GTPases in Lymphocytes

1998 • 82 citations

Proteolytic cleavage of alpha-actinin by calpain in T cells stimulated with anti-CD3 monoclonal antibody.

1996 • 78 citations

Mutations Altering the Mitochondrial-Cytoplasmic Distribution of Mod5p Implicate the Actin Cytoskeleton and mRNA 3′ Ends and/or Protein Synthesis in Mitochondrial Delivery

1995 • 78 citations

Sex-Linked Hereditary Thrombocytopenia as a Variant of Wiskott–Aldrich Syndrome

1967 • 77 citations

Linkage of the Wiskott-Aldrich syndrome with polymorphic DNA sequences from the human X chromosome.

1987 • 77 citations

A Physical Interaction between the Cell Death Protein Fas and the Tyrosine Kinase p59

1996 • 73 citations

High prevalence of nonsense, frame shift, and splice-site mutations in 16 patients with full-blown Wiskott-Aldrich syndrome

1995 • 72 citations

CD43-specific Activation of T Cells Induces Association of CD43 to Fyn Kinase

1996 • 72 citations

NATURE OF THE PLATELET DEFECT IN THE WISKOTT‐ALDRICH SYNDROME*

1972 • 71 citations

Identification of Regions of the Wiskott-Aldrich Syndrome Protein Responsible for Association with Selected Src Homology 3 Domains

1996 • 71 citations

Chemotactic Peptide-induced Activation of MEK-2, the Predominant Isoform in Human Neutrophils

1996 • 62 citations

In Vitro Reconstitution of Cortical Actin Assembly Sites in Budding Yeast

1997 • 62 citations

Localization of the gene for the Wiskott-Aldrich syndrome between two flanking markers, TIMP and DXS255, on Xp11.22–Xp11.3

1991 • 60 citations

Genetic mapping of the Wiskott-Aldrich syndrome with two highly-linked polymorphic DNA markers

1988 • 60 citations

Growth cone enrichment and cytoskeletal association of non‐receptor tyrosine kinases

1995 • 59 citations

Defective actin polymerization in EBV-transformed B-cell lines from patients with the Wiskott-Aldrich syndrome

1998 • 57 citations

Identification of N-WASP homologs in human and rat brain

1997 • 53 citations

Atypical presentation of Wiskott-Aldrich syndrome: diagnosis in two unrelated males based on studies of maternal T cell X chromosome inactivation [see comments]

1990 • 52 citations

Protein kinase C modulates actin conformation in human T lymphocytes.

1988 • 52 citations

Variable expression of WASP in B cell lines of Wiskott-Aldrich syndrome patients.

1997 • 52 citations

Tyrosine kinase signaling regulates Wiskott-Aldrich syndrome protein function, which is essential for megakaryocyte differentiation.

1997 • 51 citations

Capping of human T cell specific determinants: kinetics of capping and receptor re-expression and regulation by the cytoskeleton

1983 • 51 citations

Identification of WASP mutations, mutation hotspots and genotype-phenotype disparities in 24 patients with the Wiskott-Aldrich syndrome

1996 • 51 citations

Microfilament assembly is required for antigen-receptor-mediated activation of human B lymphocytes.

1991 • 48 citations

Lack of Platelet Response to Stimulation in the Wiskott-Aldrich Syndrome

1970 • 48 citations

Platelet survival in Wiskott-Aldrich syndrome

1966 • 47 citations

Scanning of the Wiskott-Aldrich syndrome (WAS) gene: identification of 18 novel alterations including a possible mutation hotspot at Arg86 resulting in thrombocytopenia, a mild WAS phenotype

1995 • 47 citations

The Mouse Homolog of the Wiskott–Aldrich Syndrome Protein (WASP) Gene Is Highly Conserved and Maps near the Scurfy (sf) Mutation on the X Chromosome

1995 • 46 citations

Capping of human T cell specific determinants: kinetics of capping and receptor re-expression and regulation by the cytoskeleton.

1983 • 43 citations

Actin cytoskeleton: The Arp2/3 complex gets to the point

1998 • 39 citations

Microfilament assembly modulates phospholipase C-mediated signal transduction by the TCR/CD3 in murine T helper lymphocytes.

1992 • 39 citations

Isolated X-linked thrombocytopenia in two unrelated families is associated with point mutations in the Wiskott-Aldrich syndrome protein gene

1996 • 38 citations

Wiskott-Aldrich syndrome: report of an autosomal dominant variant

1996 • 35 citations

A 5′ Regulatory Sequence Containing Two Ets Motifs Controls the Expression of the Wiskott-Aldrich Syndrome Protein (WASP) Gene in Human Hematopoietic Cells

1998 • 34 citations

Absence of Expression of the Wiskott–Aldrich Syndrome Protein in Peripheral Blood Cells of Wiskott–Aldrich Syndrome Patients

1998 • 34 citations

Platelet survival studies in Aldrich syndrome.

1966 • 32 citations

Wiskott-Aldrich syndrome and inflammatory bowel disease.

1988 • 28 citations

cAMP accumulation in T-cells inhibits anti-CD3 monoclonal antibody-induced actin polymerization

1995 • 27 citations

X-Linked Thrombocytopenia and Thrombocytopathia: Attenuated Wiskott-Aldrich Syndrome

1991 • 25 citations

Platelet Survival Studies in Aldrich Syndrome

1966 • 25 citations

The Wiskott‐Aldrich syndrome: studies of platelets, basophils and polymorphonuclear leucocytes

1986 • 25 citations

WASPbase: a database of WAS- and XLT-causing mutations

1996 • 24 citations

Antigen receptor-mediated transmembrane signaling in Wiskott-Aldrich syndrome.

1994 • 22 citations

Engagement of the T‐cell antigen receptor by anti‐CD3 monoclonal antibody causes a rapid increase in lymphocyte F‐actin

1994 • 22 citations

Effect of platelet calpain on normal T-lymphocyte CD43: hypothesis of events in the Wiskott-Aldrich syndrome

1992 • 21 citations

X-Linked Thrombocytopenic Purpura

1972 • 19 citations

Probing the gastrointestinal immune function using transgenic and knockout technology

1996 • 18 citations

Wiskott-Aldrich syndrome and related X-linked thrombocytopenia

1990 • 18 citations

Wiskott-Aldrich syndrome

1969 • 16 citations

Two sisters with clinical diagnosis of Wiskott‐Aldrich syndrome: Is the condition in the family autosomal recessive?

1995 • 16 citations

Multiple antigens are altered on T and B lymphocytes from peripheral blood and spleen of patients with Wiskott-Aldrich syndrome

1996 • 16 citations

A High-Resolution Map of Genes, Microsatellite Markers, and New Dinucleotide Repeats from UBE1 to the GATA Locus in the Region Xp11.23

1995 • 16 citations

Regulatory role of microfilaments in the induction of T4 cell proliferation and interleukin 2 production

1990 • 15 citations

Analysis of X-chromosome inactivation and presumptive expression of the Wiskott-Aldrich syndrome (WAS) gene in hematopoietic cell lineages of a thrombocytopenic carrier female of WAS

1991 • 13 citations

Evidence implicating calpain (Ca²⁺ ‐dependent neutral protease) in the destructive thrombocytopenia of the Wiskott‐Aldrich syndrome

1994 • 13 citations

Impaired Energy Metabolism in Platelets from Patients with Wiskott-Aldrich Syndrome

1989 • 11 citations

Attenuated form of Wiskott-Aldrich syndrome

1973 • 10 citations

Down-modulation of CD2 delays deletion of superantigen-responsive T cells

1998 • 8 citations