货号：557773
品牌：BD Pharmingen
规格：1EA
目录价：￥6052.00
市场价格：￥3812.76
会员价格：￥3328.60
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Wiskott-Aldrich syndrome (WAS) is an X-linked recessive immunodeficiency disease caused by mutations in the gene encoding WAS protein (WASP). The disease is characterized by a spectrum of clinical signs, including thrombocytopenia, eczema, susceptibility to opportunistic and pyogenic infections, and B-cell lymphomas associated with Epstein-Barr virus. Furthermore, patients blood cells display morphological abnormalities that can be associated with an impaired cytoskeleton. WASP is a member of a family of highly conserved proteins that link signaling pathways to the actin cytoskeleton. These members include WASP, N-WASP (neuronal), and SCAR/WAVE isoforms (Suppressor of cAMP Receptor/WASP family Verprolin-homologous protein) that share two main regions of homology: a proline-rich domain and a carboxyl terminal domain that binds to the Arp2/3 complex. The Arp2/3 complex initiates actin filament assembly in motile cells and formation of the immunological synapse between activated T lymphocytes and antigen-presenting cells. WASP is a central regulator of the actin cytoskeleton in hematopoietic cells that is itself regulated by multiple signaling pathways.The 5A5 antibody recognizes human WASP; it does not cross react with N-WASP. It has been reported to detect WASP in lysates of hematopoietic cells and cell lines, except for neutrophils, from normal donors, but not from a group of patients having mutations of the WAS gene.
- Catalog Number
- 557773
- Brand
- BD Pharmingen™
- Name
- Wiskott-Aldrich Syndrome Protein
- Alias
- WASP
- Concentration
- 0.25 mg/ml
- Size
- 0.1mg
- RegulatoryStatus
- RUO
- Clone
- 5A5
- Format
- Purified
- Isotype
- IgG2a, κ
- W.B. Positive Control
- none
- Molecular Weight
- 60 kDa
- Immunogen
- Human WASP Recombinant Protein
- Strain Host
- BALB/c
- Reactivity
- QC Testing : Human
- Applications
-
| Fluorescence microscopy | - Reported |
| Immunoprecipitation | - Reported |
| Intracellular staining (flow cytometry) | - Reported |
| Western Blot | - Routinely Tested |
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