Molecular weight (DA)
77kD
Immunogen
Purified recombinant human BTK protein fragments expressed in E.coli.
Specificity
This antibody detects endogenous levels of BTK and does not cross-react with related proteins.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification process (Immunogen)
The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
Background
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],
Function
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.,
Other name
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;IMD 1;IMD1;MGC126261;MGC126262;OTTHUMP00000063593;PSCTK 1;PSCTK1;Tyrosine protein kinase BTK;Tyrosine-protein kinase BTK;XLA
Fields
>>NF-kappa B signaling pathway;>>Osteoclast differentiation;>>Platelet activation;>>B cell receptor signaling pathway;>>Fc epsilon RI signaling pathway;>>Epstein-Barr virus infection;>>Primary immunodeficiency
Human protein sequence Database
Q06187
Mouse protein sequence database
P35991
Rat protein sequence database
Cellular localization
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.
Tissue expression
Predominantly expressed in B-lymphocytes.
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