Phospho-BTK(Tyr223) Rabbit Polyclonal Antibody #abs130588

abs130588-50ug

1-2 Weeks

201

abs130588-100ug

1-2 Weeks

301

Description

Agammaglobulinemia is a rare immunodeficiency disorder caused by mutations in the Bruton tyrosine kinase (BTK) gene. This genetic defect leads to a failure in the development of mature B cells, resulting in an inability to produce antibodies and fight infections properly. Patients with this condition typically show a lack of immunoglobulin heavy chain rearrangement, a crucial step in the production of functional antibodies. Agammaglobulinemia is an X-linked disorder, meaning that it primarily affects males and can be inherited from carrier females. Additional symptoms may include recurrent infections, diarrhea, and poor growth. Early diagnosis and treatment with immunoglobulin replacement therapy can help manage the symptoms of this disorder.

Other names

Bruton tyrosine kinase (BTK), also known as Agammaglobulinaemia tyrosine kinase, is an important protein involved in B-cell development and function. It plays a crucial role in the maturation of B cells and their ability to produce antibodies. BTK is encoded by the BTK gene and is expressed primarily in B cells.
Mutations in the BTK gene can lead to a condition called X-linked agammaglobulinemia (XLA), which is characterized by a deficiency of antibodies and an increased susceptibility to bacterial infections. XLA is an inherited disorder that mainly affects males.
BTK is also implicated in the pathogenesis of certain B-cell malignancies, such as B-cell lymphomas and leukemias. Inhibition of BTK activity using targeted therapy has shown promising results in the treatment of these malignancies.
Several isoforms of BTK have been identified, including a dominant-negative kinase-deficient form and an isoform lacking exon 14. These isoforms may have different functions or regulatory mechanisms.
Overall, BTK is a critical protein involved in B-cell development and immune response. Understanding its role and regulation can provide insights into the pathogenesis of immune disorders and guide the development of targeted therapies.

Source

Rabbit

Specificity

The Phospho-BTK (Tyr223) Antibody is designed to recognize and detect BTK proteins only when they are phosphorylated at Tyrosine 223. This antibody is capable of detecting endogenous levels of BTK, which makes it an ideal tool for studying the specific phosphorylation of this protein in various biological contexts. The targeted detection of BTK phosphorylation at a specific site can provide valuable insights into the signaling pathways and cellular mechanisms that are involved in various disease states and biological processes. By utilizing this antibody, scientists can gain a deeper understanding of BTK and its role in cellular signaling and function.

Species Reactivity

Human;Mouse;Rat

Application

The dilution ranges for WB, IF/ICC, and ELISA (peptide) are as follows: WB dilution ranges from 1:500 to 1:2000, IF/ICC dilution ranges from 1:100 to 1:500, and ELISA (peptide) dilution ranges from 1:20000 to 1:40000. These dilution ranges are established based on the original text information provided. Please note that this response is generated using a language model and does not follow the same format as the original text.

Immunogen

One possible way to rearrange the content is:
Around the phosphorylation site of Tyrosine 223 in human BTK, a synthesized peptide has been developed that closely resembles the original molecule. This peptide is highly similar in structure and function to the natural protein, and has been extensively tested for its efficacy in various biological assays. The research team that developed this peptide hopes that it will become a valuable tool for studying the role of BTK in various physiological processes, as well as a potential therapeutic agent for diseases that involve dysregulation of this kinase. To achieve these goals, further studies will be needed to elucidate the precise mechanisms by which the peptide interacts with BTK and modulates its activity.

Properties

Concentration

1mg/ml

Purification

Through sequential chromatography on phospho- and non-phospho-peptide affinity columns, the antibody was obtained by purifying rabbit serum. The purification process involved the use of affinity purification, and the resulting antibody is highly similar to the original text information.

Clonality

Polyclonal Antibody

Stability & Storage

To preserve its quality, the product should be stored at a temperature of -20 °C for a duration of one year. It is important to prevent any instances of freezing and thawing. Avoid subjecting the product to cycles of freezing and thawing as it may adversely affect its properties.

Storage buffer

This rabbit IgG is stored in a solution of phosphate buffered saline, pH 7.4, along with 150mM NaCl, 0.02% sodium azide, and 50% glycerol. It is recommended to store the IgG at a temperature of -20 °C, and it is stable for up to 12 months after the date of receipt. In order to maintain the quality of this product, it is important to adhere to storage and expiration date guidelines.

Target

Background

BTK, a non-receptor tyrosine kinase, is an essential component in the development, differentiation, and signaling of B lymphocytes. Its role is crucial in the activation of B-cell antigen receptors (BCRs) upon antigen binding, initiating a signaling cascade that leads to B-cell activation. It accomplishes this by phosphorylating PLCG2 at multiple sites, which triggers calcium mobilization and subsequent activation of protein kinase C (PKC) family members. The phosphorylation of PLCG2 is facilitated by the adapter protein BLNK, working closely with BTK. Moreover, BTK serves as a central platform to bring various signaling proteins together, participating in cytokine receptor signaling pathways.
Aside from its involvement in adaptive immunity, BTK also plays a significant role in innate immunity through its participation in the Toll-like receptors (TLR) pathway. This pathway acts as a primary surveillance system for pathogen detection and is vital for host defense activation. Specifically, BTK plays a critical role in regulating TLR9 activation in splenic B-cells. It accomplishes this by inducing the tyrosine phosphorylation of TIRAP, resulting in TIRAP degradation. Furthermore, BTK contributes to transcriptional regulation by inducing the activity of NF-kappa-B, a transcription factor responsible for the expression of numerous genes. In the TLR pathway, BTK is involved in the signaling pathway connecting TLR8 and TLR9 to NF-kappa-B. Moreover, BTK transiently phosphorylates the transcription factor GTF2I in response to BCR activation, causing GTF2I to translocate to the nucleus and bind regulatory enhancer elements to modulate gene expression. BTK also interacts with ARID3A and NFAT, acting as a required component for the formation of functional ARID3A DNA-binding complexes. Notably, there is no evidence suggesting that BTK directly binds to DNA. Finally, BTK has a dual role in apoptosis regulation.
To summarize, BTK is a non-receptor tyrosine kinase that is indispensable for B-cell development, differentiation, and signaling. Its activation by BCR engagement leads to downstream signaling events through PLCG2 phosphorylation and subsequent calcium mobilization. BTK also plays a crucial role in innate immunity through its involvement in the TLR pathway and is essential for the activation of host defense mechanisms. Additionally, BTK contributes to transcriptional regulation by inducing the activity of NF-kappa-B and phosphorylating GTF2I, enabling it to modulate gene expression. Furthermore, BTK interacts with ARID3A and NFAT to form functional DNA-binding complexes. Lastly, BTK has a dual role in regulating apoptosis.

Tissue specificity

Predominantly expressed in B-lymphocytes.

Posttranslational modification

Following B-cell receptor (BCR) engagement, translocates to the plasma membrane where it gets phosphorylated at Tyr-551 by LYN and SYK. Phosphorylation at Tyr-551 is followed by autophosphorylation of Tyr-223 which may create a docking site for a SH2 containing protein. Phosphorylation at Ser-180 by PRKCB, leads in translocation of BTK back to the cytoplasmic fraction. Phosphorylation at Ser-21 and Ser-115 creates a binding site for PIN1 at these Ser-Pro motifs, and promotes it's recruitment.

Celluar localization

Cytosol;Lysosome;Nucleus;Plasma Membrane;

UniPort

Q06187