概述

描述

  • 性质Synthetic
  • 氨基酸序列
    • 种属Human

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技术指标

Our Abpromise guarantee covers the use of ab39736 in the following tested applications.

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

  • 应用

    Blocking

    Western blot

  • 形式Liquid
  • 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 or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: 0.02% Sodium Azide
    Constituents: PBS, 0.1% bovine serum albumin, pH 7.2

常规信息

  • 别名
    • Agammaglobulinaemia tyrosine kinase
    • AGMX 1
    • AGMX1
    • AT
    • ATK
    • B cell progenitor kinase
    • B-cell progenitor kinase
    • BPK
    • Bruton agammaglobulinemia tyrosine kinase
    • Bruton tyrosine kinase
    • Bruton’s Tyrosine Kinase
    • Btk
    • BTK_HUMAN
    • dominant-negative kinase-deficient Brutons tyrosine kinase
    • IMD 1
    • IMD1
    • MGC126261
    • MGC126262
    • OTTHUMP00000063593
    • PSCTK 1
    • PSCTK1
    • truncated Bruton agammaglobulinemia tyrosine kinase
    • Tyrosine protein kinase BTK
    • Tyrosine-protein kinase BTK
    • tyrosine-protein kinase BTK isoform (lacking exon 14
    • XLA
    see all
  • 功能Plays a crucial role in B-cell ontogeny. Transiently phosphorylates GTF2I on tyrosine residues in response to B-cell receptor cross-linking. Required for the formation of functional ARID3A DNA-binding complexes.
  • 疾病相关Defects in BTK are the cause of X-linked agammaglobulinemia (XLA) [MIM:300755]; also known as X-linked agammaglobulinemia type 1 (AGMX1) or immunodeficiency type 1 (IMD1). XLA is a humoral immunodeficiency disease which results in developmental defects in the maturation pathway of B-cells. Affected boys have normal levels of pre-B-cells in their bone marrow but virtually no circulating mature B-lymphocytes. This results in a lack of immunoglobulins of all classes and leads to recurrent bacterial infections like otitis, conjunctivitis, dermatitis, sinusitis in the first few years of life, or even some patients present overwhelming sepsis or meningitis, resulting in death in a few hours. Treatment in most cases is by infusion of intravenous immunoglobulin.
    Defects in BTK may be the cause of X-linked hypogammaglobulinemia and isolated growth hormone deficiency (XLA-IGHD) [MIM:307200]; also known as agammaglobulinemia and isolated growth hormone deficiency or Fleisher syndrome or isolated growth hormone deficiency type 3 (IGHD3). In rare cases XLA is inherited together with isolated growth hormone deficiency (IGHD).
  • 序列相似性Belongs to the protein kinase superfamily. Tyr protein kinase family. TEC subfamily.
    Contains 1 Btk-type zinc finger.
    Contains 1 PH domain.
    Contains 1 protein kinase domain.
    Contains 1 SH2 domain.
    Contains 1 SH3 domain.
  • 翻译后修饰Autophosphorylated on Tyr-223 and Tyr-551. Phosphorylation of Tyr-223 may create a docking site for a SH2 containing protein.
  • 细胞定位Cytoplasm. Membrane. Nucleus.
  • Information by UniProt

Human BTK peptide (ab39736)参考文献

ab39736 has not yet been referenced specifically in any publications.

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