| 產(chǎn)品編號 | bs-13681R |
| 英文名稱(chēng) | WASP Rabbit pAb |
| 中文名稱(chēng) | 濕疹血小板減少伴免疫缺陷綜合征相關(guān)蛋白抗體 |
| 別 名 | Eczema thrombocytopenia; IMD2; SCNX; THC; THC1; Thrombocytopenia 1(X linked); U42471; Was; WASp; WASP_HUMAN; Wiskott Aldrich syndrome(eczema thrombocytopenia); Wiskott Aldrich syndrome; Wiskott Aldrich syndrome protein; Wiskott-Aldrich syndrome protein. |
| 研究領(lǐng)域 | 細胞生物 免疫學(xué) 細胞分化 細胞骨架 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應 | Human,Mouse (predicted: Rat,Rabbit,Cow) |
| 產(chǎn)品應用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 53 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human WASP: 101-200/502 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
The Wiskott-Aldrich syndrome (WAS) is a disorder that results from a monogenic defect that has been mapped to the short arm of the X chromosome. WAS is characterized by thrombocytopenia, eczema, defects in cell-mediated and humoral immunity and a propensity for lymphoproliferative disease. The gene that is mutated in the syndrome encodes a proline-rich protein of unknown function designated WAS protein (WASP). A clue to WASP function came from the observation that T cells from affected males had an irregular cellular morphology and a disarrayed cytoskeleton suggesting the involvement of WASP in cytoskeletal organization. Close examination of the WASP sequence revealed a putative Cdc42/Rac interacting domain, homologous with those found in PAK65 and ACK. Subsequent investigation has shown WASP to be a true downstream effector of Cdc42. 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. Subunit: Interacts with NCK1 (via SH3 domains). Interacts with CDC42, RAC, NCK, HCK, FYN, SRC kinase FGR, BTK, ABL1, PSTPIP1, WIP, and to the p85 subunit of PLC-gamma. Binds the Arp2/3 complex. Interacts (via C-terminus) with ALDOA. Interacts with E.coli effector protein EspF(U). Subcellular Location: Cytoplasm; cytoskeleton. Tissue Specificity: Expressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen Post-translational modifications: Phosphorylated at Tyr-291 by FYN and HCK, inducing WAS effector activity after TCR engagement. Phosphorylation at Tyr-291 enhances WAS activity in promoting actin polymerization and filopodia formation. 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. 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. Defects in WAS are a cause of neutropenia severe congenital X-linked (XLN) [MIM:300299]. XLN is an immunodeficiency syndrome characterized by recurrent major bacterial infections, severe congenital neutropenia, and monocytopenia. Similarity: Contains 1 CRIB domain. Contains 1 WH1 domain. Contains 1 WH2 domain. SWISS: P42768 Gene ID: 7454 Database links: Entrez Gene: 7454 Human Entrez Gene: 22376 Mouse Omim: 300392 Human SwissProt: P42768 Human SwissProt: P70315 Mouse Unigene: 2157 Human |
| 產(chǎn)品圖片 |
Sample:
Jurkat(Human) Cell Lysate at 30 ug
Raji(Human) Cell Lysate at 30 ug
Primary: Anti-WASP (bs-13681R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 53 kD
Sample:
U937(Human) Cell Lysate at 30 ug
Primary: Anti-WASP (bs-13681R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 53 kD
Sample:
Spleen (Mouse) Lysate at 40 ug
Raji Cell (Human) Lysate at 30 ug
Primary: Anti- WASP (bs-13681R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 50 kD
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