Product Description

Btk Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES4076-50, ES4076-100

Lead times: approximately 7-10 business days, if manufacturer has product in stock

Manufacturer/Ship Location: China

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],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IHC, IF, ELISA

Alternate Names: BTK; AGMX1; ATK; BPK; Tyrosine-protein kinase BTK; Agammaglobulinaemia tyrosine kinase; ATK; B-cell progenitor kinase; BPK; Bruton tyrosine kinase

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from Btk . at AA range: 490-570

Isotype: IgG

Target: Btk,

Specificity: Btk Polyclonal Antibody detects endogenous levels of Btk protein only when non-phosphorylation at Y550.

Dilution: Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/20000. Not yet tested in other applications.

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formulation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Expression: Predominantly expressed in B-lymphocytes.

Observed Band (KD): 76kD

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

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.

Subcellular Location: 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.

Storage and Stability: -20°C/1 year

Gene Name: BTK

Protein Name: Tyrosine-protein kinase BTK

Human Gene ID: 695

Human SwissProt Number: Q06187

Mouse Gene ID: 12229

Mouse SwissProt Number: P35991

Research Use Only