Applications: | IHC-P, IF, WB |
Species Reactivity: | Human |
Isotype: | Rabbit Ig |
Purification: | Purified Rabbit Polyclonal Antibody |
Description: | 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. |
Further Information: | This BTK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 209-239 amino acids from the Central region of human BTK. |
Synonym: | Tyrosine-protein kinase BTK, Agammaglobulinemia tyrosine kinase, ATK, B-cell progenitor kinase, BPK, Bruton tyrosine kinase, BTK, AGMX1, ATK, BPK |
Ascension Number: | Q06187 |
NCBI EntrezGene: | 695 |
Shipping Conditions: | Ship on cold packs |
Storage: | Maintain refrigerated at 2-8 C for up to 2 weeks. For long term storage store at -20 C in small aliquots to prevent freeze-thaw cycles. |
Usage: | Research use only |