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Rabbit Anti-WASP/Gold Conjugated antibody (bs-13681R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-13681R-Gold
英文名稱1 Rabbit Anti-WASP/Gold Conjugated antibody
中文名稱 膠體金標記的濕疹血小板減少伴免疫缺陷綜合征相關蛋白抗體
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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 細胞生物  免疫學  細胞分化  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse,  (predicted: Rat, Cow, Rabbit, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human WASP
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 background:
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.

Database links:

Entrez Gene: 7454 Human

Entrez Gene: 22376 Mouse

Omim: 300392 Human

SwissProt: P42768 Human

SwissProt: P70315 Mouse

Unigene: 2157 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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