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

Derry, Jonathan M.J.; Wiedemann, Philipp; Blair, Patrick J.; Wang, Yu-Ker; Kerns, Julie A.; Lemahieu, Vanessa; Godfrey, Virginia; Wilkinson, Erby; Francke, Uta

doi:10.1006/geno.1995.9979

Cited by 42 publications

(26 citation statements)

References 2 publications

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“…Each gene contains 12 exons, nearly identical in size and composition, which span Ͻ10 kb of genomic DNA. WASP and mouse WASP share 86% amino acid identity deduced from cDNA sequence, and most studies have suggested that their expression is limited to the hemopoietic cell lineage that is consistent with the cell type affected in WAS patients (3)(4)(5)(6)(7).…”

Section: W Iskott-aldrich Syndrome (Was)mentioning

confidence: 92%

“…A pleckstrin homology (PH) domain at the N-terminal region of WASP is a divergent protein module of ϳ100 amino acids found in many proteins involved in signal transduction. There is evidence that PH domains are capable of binding phosphatidylinositol (4,5) bisphosphate (PI(4,5)P2) and regulating protein interactions beneath the cell membrane (9 -13) despite low amino acid sequence homology among the PH domains. A prolinerich region binds to the Src homology 3 domain of the adaptor Nck, Grb2 (14,15), and of several kinases, including Fyn, Tec, Itk, and Btk (16 -18).…”

Section: W Iskott-aldrich Syndrome (Was)mentioning

confidence: 99%

“…The T7 tag (MASMTGGQQMG) (30,31) was generated as follows: coding linker, containing a KpnI site at the 5Ј end of the linker (5Ј-CCATGGCCAGCA TGACCGGCGGCCAGCAGATGGGCG-3Ј), and noncoding linker, containing a BamHI site at the end of the linker (5Ј-GATCCGCCCATCTGC TGGCCGCCGGTCATGCTGGCCATGGGTAC-3Ј), were annealed and inserted into a KpnI/BamHI site of pCR3/WASP exon 1-5 (to be generated fusion protein with T7 tag and WASP exon [1][2][3][4][5]. This construct was designated as pCR/T7-WASP15.…”

Section: Plasmid Constructionmentioning

confidence: 99%

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Overexpression of the Wiskott-Aldrich Syndrome Protein N-Terminal Domain in Transgenic Mice Inhibits T Cell Proliferative Responses Via TCR Signaling Without Affecting Cytoskeletal Rearrangements

Sato

Tsuji

Gotoh

et al. 2001

The Journal of Immunology

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Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder characterized by thrombocytopenia with small platelets, severe eczema, and recurrent infections due to defects in the immune system. The disease arises from mutations in the gene encoding the WAS protein (WASP), which plays a role as an adaptor molecule in signal transduction accompanied by cytoskeletal rearrangement in T cells. To investigate the functional domain of WASP, we developed transgenic mice overexpressing the WASP N-terminal region (exon 1–5) including the Ena/VASP homology 1 (pleckstrin homology/WASP homology 1) domain, in which the majority of mutations in WAS patients have been observed. WASP transgenic mice develop and grow normally under the specific pathogen-free environment, and showed normal lymphocyte development. However, proliferative responses and cytokine production induced by TCR stimulation were strongly inhibited in transgenic mice, whereas Ag receptor capping and actin polymerization were normal. These findings suggest that overexpressed Ena/VASP homology 1 (pleckstrin homology/WASP homology 1) domain of WASP inhibits the signaling from TCR without coupling of cytoskeletal rearrangement. WASP transgenic mice shown here could be valuable tools for further understanding the WASP-mediated processes.

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Section: W Iskott-aldrich Syndrome (Was)mentioning

confidence: 92%

Section: W Iskott-aldrich Syndrome (Was)mentioning

confidence: 99%

Section: Plasmid Constructionmentioning

confidence: 99%

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Overexpression of the Wiskott-Aldrich Syndrome Protein N-Terminal Domain in Transgenic Mice Inhibits T Cell Proliferative Responses Via TCR Signaling Without Affecting Cytoskeletal Rearrangements

Sato

Tsuji

Gotoh

et al. 2001

The Journal of Immunology

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“…Although Src activation and cortactin tyrosine phosphorylation are coincident with thrombin-mediated platelet activation following binding to a IIB b 3 integrin (Fox et al, 1993), there is also evidence that the hematopoieticspec®c kinase Syk plays an important role in cortactin tyrosine phosphorylation during platelet maturation and activation. Syk is homologous to the lymphocyte- Derry et al (1995) (WASP), Fukuoka et al (1997) (N-WASP) and Bear et al (1998) (Scar-1). (c) Src tyrosine phosphorylation sites on cortactin conform to a consensus SH2-binding motif.…”

Section: Cortactin As a Target For Tyrosine And Serine/threonine Protmentioning

confidence: 99%

Cortactin: coupling membrane dynamics to cortical actin assembly

2001

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Exposure of cells to a variety of external signals causes rapid changes in plasma membrane morphology. Plasma membrane dynamics, including membrane ru e and microspike formation, fusion or ®ssion of intracellular vesicles, and the spatial organization of transmembrane proteins, is directly controlled by the dynamic reorganization of the underlying actin cytoskeleton. Two members of the Rho family of small GTPases, Cdc42 and Rac, have been well established as mediators of extracellular signaling events that impact cortical actin organization. Actin-based signaling through Cdc42 and Rac ultimately results in activation of the actin-related protein (Arp) 2/3 complex, which promotes the formation of branched actin networks. In addition, the activity of both receptor and non-receptor protein tyrosine kinases along with numerous actin binding proteins works in concert with Arp2/3-mediated actin polymerization in regulating the formation of dynamic cortical actin-associated structures. In this review we discuss the structure and role of the cortical actin binding protein cortactin in Rho GTPase and tyrosine kinase signaling events, with the emphasis on the roles cortactin plays in tyrosine phosphorylation-based signal transduction, regulating cortical actin assembly, transmembrane receptor organization and membrane dynamics. We also consider how aberrant regulation of cortactin levels contributes to tumor cell invasion and metastasis. Oncogene (2001) 20, 6418 ± 6434.

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“…This did not prove to be the case, however, as the mouse WASP gene was not mutated in the scurfy mouse (Derry et al 1995b). Other candidate genes mutated in scurfy or tattered mice (Merrell et al 1995) will become evident as this interval is sequenced.…”

Section: Syntenic Regions In the Mousementioning

confidence: 97%

Long-range map of a 3.5-Mb region in Xp11.23-22 with a sequence-ready map from a 1.1-Mb gene-rich interval.

Schindelhauer

Hellebrand²,

Grimm

et al. 1996

Genome Res.

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Most of the yeast artificial chromosomes (YACs} isolated from the Xp11.23-22 region have shown instability and chimerism and are not a reliable resource for determining physical distances. We therefore constructed a long-range pulsed-field gel electrophoresis map that encompasses -3.5 Mb of genomic DNA between the loci TIMP and DXSI46 including a CpG-rich region around the WASP and TFE-3 gene loci. A combined YAC-cosmid contig was constructed along the genomic map and was used for fine-mapping of 15 polymorphic microsatellites and 30 expressed sequence tags (ESTs} or sequence transcribed sites {STSs}, revealing the following order: teI~SYN-TIMPHDXS426-ELKI~ZNF(CAJn

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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

Cited by 42 publications

References 2 publications

Overexpression of the Wiskott-Aldrich Syndrome Protein N-Terminal Domain in Transgenic Mice Inhibits T Cell Proliferative Responses Via TCR Signaling Without Affecting Cytoskeletal Rearrangements

Overexpression of the Wiskott-Aldrich Syndrome Protein N-Terminal Domain in Transgenic Mice Inhibits T Cell Proliferative Responses Via TCR Signaling Without Affecting Cytoskeletal Rearrangements

Cortactin: coupling membrane dynamics to cortical actin assembly

Long-range map of a 3.5-Mb region in Xp11.23-22 with a sequence-ready map from a 1.1-Mb gene-rich interval.

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