概述

描述

  • 性质
    Synthetic
  • 氨基酸序列
    • 种属
      Human

技术指标

Our Abpromise guarantee covers the use of ab42784 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 生物活性
    This sequence is highly homologous with a similar region in Human N WASP.
  • 应用

    Blocking

    ELISA

  • 纯度
    > 90 % SDS-PAGE.

  • 形式
    Liquid
  • 补充说明
    This sequence is highly homologous with a similar region in Human N WASP.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

    Preservative: 0.05% Sodium Azide
    Constituents: PBS

常规信息

  • 别名
    • Eczema thrombocytopenia
    • IMD2
    • SCNX
    • THC
    • THC1
    • Thrombocytopenia 1 (X linked)
    • U42471
    • Was
    • WASp
    • WASP_HUMAN
    • Wiskott Aldrich syndrome
    • Wiskott Aldrich syndrome (eczema thrombocytopenia)
    • Wiskott Aldrich syndrome protein
    • Wiskott-Aldrich syndrome protein
    see all
  • 功能
    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.
  • 组织特异性
    Expressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen.
  • 疾病相关
    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.
  • 序列相似性
    Contains 1 CRIB domain.
    Contains 1 WH1 domain.
    Contains 1 WH2 domain.
  • 结构域
    The WH1 (Wasp homology 1) domain may bind a Pro-rich ligand.
    The CRIB (Cdc42/Rac-interactive-binding) region binds to the C-terminal WH2 domain in the autoinhibited state of the protein. Binding of Rho-type GTPases to the CRIB induces a conformation change and leads to activation.
  • 细胞定位
    Cytoplasm > cytoskeleton.
  • Information by UniProt

文献

ab42784 has not yet been referenced specifically in any publications.

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