The Wiskott-Aldrich syndrome

Israel Medical Association Journal

Volumn 4, Issue 5, 2002, Pages 379-384

  Ochs, Hans D ^a  

^a UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

Author keywords

Antibiotic therapy; Bone marrow transplantation; Immunodeficiency; Intravenous immunoglobulin; Malignancy; Small platelets; Wiskott Aldrich syndrome; X linked thrombocytopenia

Indexed keywords

ANTIBIOTIC AGENT; WISKOTT ALDRICH SYNDROME PROTEIN;

ANTIBIOTIC THERAPY; BONE MARROW TRANSPLANTATION; CELL SIZE; CLINICAL FEATURE; CYTOSKELETON; DIAGNOSTIC PROCEDURE; DISEASE ASSOCIATION; DISEASE CARRIER; DISEASE SEVERITY; ECZEMA; HUMAN; IMMUNE DEFICIENCY; MALIGNANT TRANSFORMATION; MOLECULAR DYNAMICS; NUCLEOTIDE SEQUENCE; PRENATAL DIAGNOSIS; PROGNOSIS; PROTEIN ANALYSIS; PROTEIN DETERMINATION; PROTEIN DOMAIN; PROTEIN FUNCTION; PROTEIN STRUCTURE; REVIEW; SCORING SYSTEM; SIGNAL TRANSDUCTION; SKIN MANIFESTATION; THROMBOCYTE DYSFUNCTION; THROMBOCYTOPENIA; WISKOTT ALDRICH SYNDROME; X CHROMOSOME LINKED DISORDER;

HUMANS; PROTEINS; WISKOTT-ALDRICH SYNDROME; WISKOTT-ALDRICH SYNDROME PROTEIN;

EID: 0036252130     PISSN: 15651088     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Review

References (37)

1. Familiärer, angeborener Morbus Werlhofii?
2. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea
3. A multiinstitutional survey of the Wiskott-Aldrich syndrome
4. X-linked thrombocytopenia and Wiskott-Aldrich syndrome are allelic diseases with mutations in the WASP gene
5. The Wiskott-Aldrich syndrome and X-linked congenital thrombocytopenia are caused by mutations of the same gene
6. Isolation of a novel gene mutated in Wiskott-Aldrich syndrome
7. WASPbase: A database of WAS- and XLT-causing mutations
8. Scarl and the related Wiskott-Aldrich syndrome protein WASP regulate the actin cytoskeleton through the Arp2/3 complex
9. Wiskott-Aldrich syndrome/X-linked thrombocytopenia: WASP mutations, protein expression, and phenotype
10. The Wiskott-Aldrich syndrome: Studies of lymphocytes, granulocytes, and platelets
11. T cells of patients with the Wiskott-Aldrich syndrome have a restricted defect in proliferative responses
12. Actin cytoskeletal function is spared, but apoptosis is increased, in WAS patient hematopoietic cells
13. Defective actin reorganization and polymerization of Wiskott-Aldrich T cells in response to CD3-mediated stimulation
14. Wiskott-Aldrich syndrome protein is necessary for efficient IgG-mediated phagocytosis
15. Cutting edge: The Wiskott-Aldrich syndrome protein is required for efficient phagocytosis of apoptotic cells
16. Regulation of the polarization of T cells toward antigen-presenting cells by Ras-related GTPase CDC42
17. Monocytes from Wiskott-Aldrich patients display reduced chemotaxis and lack of cell polarization in response to monocytes chemoattractant protein-1 and formylmethionyl-leucyl-phenylalanine
18. Intrinsic dendritic cell abnormalities in Wiskott-Aldrich syndrome
19. Is Wiskott-Aldrich syndrome a cell trafficking disorder?
20. Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia
21. Intravenous immunoglobulin, splenectomy, and antibiotic prophylaxis in Wiskott-Aldrich syndrome
22. Wiskott-Aldrich syndrome: Qualitative platelet defects and short platelet survival
23. Tyrosine kinase signaling regulates Wiskott-Aldrich syndrome protein function, which is essential for megakaryocyte differentiation
24. Nonrandom inactivation of the X chromosome in early lineage hematopoietic cells in carriers of Wiskott-Aldrich syndrome
25. Impact of donor type on outcome of bone marrow transplantation for Wiskott-Aldrich syndrome: Collaborative study of the International Bone Marrow Transplant Registry and the Nation Marrow Donor Program
26. N-WASP, a novel actin-depolymerizing protein, regulates the cortical cytoskeletal rearrangement in a PIP2-dependent manner downstream of tyrosine kinases
27. Wiskott-Aldrich syndrome protein, a novel effector for the GTPase CDC42Hs, is implicated in actin polymerization
28. WIP, a protein associated with Wiskott-Aldrich syndrome protein, induces actin polymerization and redistribution in lymphoid cells
29. A complex of N-WASP and WIP integrates signaling cascades that lead to actin polymerization
30. Rac and cdc42 induce actin polymerization and G1 cell cycle progression independently of p65PAK and the JNK/SAPK MAP kinase cascade
31. Autoinhibition and activation mechanisms of the Wiskott-Aldrich syndrome protein
32. Signaling to actin: The Cdc42-N-WASP-Arp2/3 connection
33. The interaction between N-WASP and the Arp2/3 complex links Cdc42-dependent signals to actin assembly
34. Involvement of Wiskott-Aldrich syndrome protein in B cell cytoplasmic tyrosine kinase pathway
35. Collagen induces tyrosine phosphorylation of Wiskott-Aldrich syndrome protein in human platelets
36. Constitutively activating mutation in WASP causes X-linked severe congenital neutropenia
37. Spontaneous apoptosis in lymphocytes from patients with Wiskott-Aldrich syndrome: Correlation of accelerated cell death and attenuated Bcl-2 expression