Hu WASP Pure 5A5 100ug
货号:
557773 基本售价:
5092.0 元 规格:
100ug
产品信息
概述货号 | 557773 |
描述 | Wiskott-Aldrichsyndrome (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 cAMPReceptor/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. |
别名 | WASP |
同种亚型 | IgG2a,κ |
反应种属 | Human (QC Testing) |
来源宿主 | Mouse |
使用方法 | WB |
性能供应商 | BD Pharmingen |
免疫原 | Human WASP Recombinant Protein |
存放说明 | The monoclonal antibody was purified from tissue culture supernatant or ascites by affinity chromatography.Store undiluted at 4°C.
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存储溶液 | Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide. |
浓度 | 0.25 mg/ml |