产品详情
产品名称WAS Antibody
来源种属Rabbit
克隆性Polyclonal
亚型IgG
纯化Affinity purification
应用WB
种属反应性Human
特异性The antibody detects endogenous level of total WAS protein.
免疫原类型Recombinant Protein
免疫原描述Recombinant fusion protein of human WASPP (NP_000368.1).
基因/蛋白名称WAS
别名WAS;IMD2;SCNX;THC;THC1;WASP;WASPA
数据库入口号Uniprot:P42768
GeneID:7454
UniprotP42768
Gene ID7454
实际分子量62kDa
浓度1.0mg/ml
配方PBS with 0.02% sodium azide,50% glycerol,pH7.3.
保存Store at -20˚C. Avoid freeze / thaw cycles.
应用详情
WB 1:500 - 1:2000
背景
The Wiskott-Aldrich syndrome (WAS) family of proteins share similar domain structure, and are involved in transduction of signals from receptors on the cell surface to the actin cytoskeleton. The presence of a number of different motifs suggests that they are regulated by a number of different stimuli, and interact with multiple proteins. Recent studies have demonstrated that these proteins, directly or indirectly, associate with the small GTPase, Cdc42, known to regulate formation of actin filaments, and the cytoskeletal organizing complex, Arp2/3. Wiskott-Aldrich syndrome is a rare, inherited, X-linked, recessive disease characterized by immune dysregulation and microthrombocytopenia, and is caused by mutations in the WAS gene. The WAS gene product is a cytoplasmic protein, expressed exclusively in hematopoietic cells, which show signalling and cytoskeletal abnormalities in WAS patients. A transcript variant arising as a result of alternative promoter usage, and containing a different 5' UTR sequence, has been described, however, its full-length nature is not known.