Total WASP Cell-Based Colorimetric ELISA Kit
- Catalog No.:KA4322C
- Applications:ELISA
- Reactivity:Human;Mouse;
- Gene Name:
- WAS
- Human Gene Id:
- 7454
- Human Swiss Prot No:
- P42768
- Mouse Swiss Prot No:
- P70315
- Storage Stability:
- 2-8°C/6 months
- Other Name:
- Wiskott-Aldrich syndrome protein (WASp)
- Detection Method:
- Colorimetric
- Background:
- disease:Defects in WAS are a cause of X-linked severe congenital neutropenia (XLN) [MIM:300299]. XLN is an X-linked immunodeficiency syndrome characterized by recurrent major bacterial infections, severe congenital neutropenia, and monocytopenia.,disease:Defects in WAS are the cause of thrombocytopenia type 1 (THC1) [MIM:313900]. Thrombocytopenia is defined by a decrease in the number of platelets in circulating blood, resulting in the potential for increased bleeding and decreased ability for clotting.,disease:Defects in WAS are the cause of Wiskott-Aldrich syndrome (WAS) [MIM:301000]; also known as eczema-thrombocytopenia-immunodeficiency syndrome. WAS is an X-linked recessive immunodeficiency characterized by eczema, thrombocytopenia, recurrent infections, and bloody diarrhea. Death usually occurs before age 10.,domain:The CRIB (Cdc42/Rac-interactive-binding) region binds to the C-terminal WH2 domain in the autoinhibited state of the protein. Binding of Rho-type GTPases to the CRIB induces a conformation change and leads to activation.,domain:The WH1 (Wasp homology 1) domain may bind a Pro-rich ligand.,function:Effector protein for Rho-type GTPases, providing a link with the Arp2/3 complex that regulates the structure and dynamics of the actin cytoskeleton. Important for efficient actin polymerization. Possible regulator of lymphocyte and platelet function.,online information:WAS mutation db,online information:Wiskott-Aldrich syndrome protein entry,similarity:Contains 1 CRIB domain.,similarity:Contains 1 WH1 domain.,similarity:Contains 1 WH2 domain.,subunit:Binds to CDC42, RAC, NCK, FYN, SRC kinase FGR, BTK, ABL, PSTPIP1, WIP, and to the p85 subunit of PLC-gamma. Binds the Arp2/3 complex.,tissue specificity:Expressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen.,
- Function:
- cell activation, protein complex assembly, defense response, immune response, cytoskeleton organization, actin filament organization, ectoderm development, blood coagulation, hemostasis, actin polymerization or depolymerization, epidermis development, response to wounding, vesicle-mediated transport, endosome transport,actin filament-based process, actin cytoskeleton organization, actin filament polymerization, actin filament-based movement, cytoskeleton-dependent intracellular transport, cellular macromolecular complex subunit organization,cellular macromolecular complex assembly, wound healing, T cell activation, cellular protein complex assembly,macromolecular complex subunit organization, leukocyte activation, lymphocyte activation, intracellular transport,coagulation, regulation of body fluid levels, protein polymerization, macromolecular complex assembly, protein complex biogenesis
- Subcellular Location:
- Cytoplasm, cytoskeleton . Nucleus .
- Expression:
- Expressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen.
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