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Rabbit Anti-WASP  antibody (bs-13681R)  
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-13681R
英文名称 Rabbit Anti-WASP  antibody
中文名称 湿疹血小板减少伴免疫缺陷综合征相关蛋白抗体
别    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.  
研究领域 细胞生物  免疫学  细胞分化  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,Mouse (predicted: Rat,Rabbit,Cow)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 53 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human WASP: 101-200/502 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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



产品图片
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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