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Btk Rabbit Polyclonal Antibody  Cite:0    分享

货号： IM56633
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  • 种属：H，M，R
  • 用途：WB，ELISA，IHC-P，IF-P，IF-F，IF-ICC
规格:
50μl 100μl
单位:
支
单价:￥1500.00
产品简介
储存与保存
注意事项
产品简介
  • 产品名称
    Btk Rabbit Polyclonal Antibody
  • 别名
    BTK; AGMX1; ATK; BPK; Tyrosine-protein kinase BTK; Agammaglobulinaemia tyrosine kinase; ATK; B-cell progenitor kinase; BPK; Bruton tyrosine kinase
  • 类别
    抗体产品
  • 基因名称
    BTK
  • 蛋白名称
    Tyrosine-protein kinase BTK
  • Clonality
    Polyclonal
  • 推荐应用
    WB,IHC-P,IF-P,IF-F,IF-ICC,ELISA
  • 稀释度
    WB 1:500-1:2000, IHC-P 1:100-1:300, ELISA 1:20000, IF-P/IF-F/IF-ICC 1:50-200
  • 反应种属
    Human,Mouse,Rat
  • 浓度
    1 mg/ml
  • 存储缓冲液
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
  • Human Gene ID
    695
  • Human Swissprot No.
    Q06187
  • Mouse Gene ID
    12229
  • Mouse Swissprot No.
    P35991
  • 免疫原
    Synthesized peptide derived from Btk . at AA range: 490-570
  • 特异性
    Btk Polyclonal Antibody detects endogenous levels of Btk protein only when non-phosphorylation at Y550.
  • 预测分子量
    76kDa
  • 宿主
    Rabbit
  • 同种型
    Rabbit,IgG
  • 背景介绍
    The protein encoded by this gene plays a crucial role in B-cell development. Mutations in this gene cause X-linked agammaglobulinemia type 1, which is an immunodeficiency characterized by the failure to produce mature B lymphocytes, and associated with a failure of Ig heavy chain rearrangement. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2013],
  • 组织表达
    Predominantly expressed in B-lymphocytes.
  • 细胞定位
    Cytoplasm. Cell membrane; Peripheral membrane protein. Nucleus. In steady state, BTK is predominantly cytosolic. Following B-cell receptor (BCR) engagement by antigen, translocates to the plasma membrane through its PH domain. Plasma membrane localization is a critical step in the activation of BTK. A fraction of BTK also shuttles between the nucleus and the cytoplasm, and nuclear export is mediated by the nuclear export receptor CRM1.
  • 信号通路
    B_Cell_Antigen;Fc epsilon RI;Primary immunodeficiency;
  • 功能
    catalytic activity:ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate.,cofactor:Binds 1 zinc ion per subunit.,disease:Defects in BTK are the cause of X-linked agammaglobulinemia (XLA) [MIM:300755]; also called 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.,disease: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).,enzyme regulation:Inhibited by IBTK. Activated by phosphorylation.,function: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.,online information:BTK mutation db,PTM:Autophosphorylated on Tyr-223 and Tyr-551. Phosphorylation of Tyr-223 may create a docking site for a SH2 containing protein.,similarity:Belongs to the protein kinase superfamily. Tyr protein kinase family.,similarity:Belongs to the protein kinase superfamily. Tyr protein kinase family. TEC subfamily.,similarity:Contains 1 Btk-type zinc finger.,similarity:Contains 1 PH domain.,similarity:Contains 1 protein kinase domain.,similarity:Contains 1 SH2 domain.,similarity:Contains 1 SH3 domain.,subunit:Binds GTF2I through the PH domain. Interacts with SH3BP5 via the SH3 domain. Interacts with IBTK via its PH domain. Interacts with GTF2I and ARID3A.,
  • 纯化
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存与保存

1.保存：-20℃，Avoid freeze/thaw cycles

2.有效期：1年

注意事项

1. 本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。 

2. 为了您的安全和健康，请穿好实验服并佩戴一次性手套和口罩操作。 

3. 实验结果可由多种因素影响，相关处理只限于产品本身，不涉及其他赔偿。 

 

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