BTK plays a crucial role in B-cell ontogeny. This protein transiently phosphorylates GTF2I on tyrosine residues in response to B-cell receptor cross-linking. Defects in BTK are the cause of X-linked agammaglobulinemia type 1 (XLA). 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 or fatal sepsis or meningitis within the first years of life.
| Target |
BTK |
| Reactivity |
Human |
| Host |
Rabbit |
| Clonality |
Polyclonal |
| Tested Applications |
WB, IHC, IF/ICC |
| Recommended dilutions |
Optimal dilutions/concentrations should be determined by the end user. |
| Immunogen |
KLH-conjugated synthetic peptide between 209-239 amino acids from the Central region of human BTK. |
| Purification |
Purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS. |
| Isotype |
IgG |
| Conjugation |
Unconjugated |
| Storage |
Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Swiss Prot |
Q06187
|
| Buffer |
PBS containing 0.09% sodium azide. |
| UNSPSC Code |
12352203 |
| Availability |
Shipped within 5-10 working days. |
| Note |
This product is for research use only. |