Content Loading...
Content Loading...
Product Images
Sample enzyme activity data. See certificate of analysis for lot specific activity data.
BTK, active
Description:
BTK, active
Trade Name:
Upstate (Millipore)
Qty/Pk:
10 µg
Product Overview:
N-terminal His6-tagged, recombinant, full-length, human BTK
Key Applications:
Kinase Assay
Usage Statement:
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Entrez Gene Summary:
Defects in the Bruton tyrosine kinase (BTK) gene cause Agammaglobulinemia. Agammaglobulinemia is an X-linked immunodeficiency characterized by failure to produce mature B lymphocyte cells and associated with a failure of Ig heavy chain rearrangement.
UniProt Summary:
FUNCTION: SwissProt: Q06187 # Plays a crucial role in B-cell ontogeny. Transiently phosphorylates GTF2I on tyrosine residues in response to B-cell receptor cross-linking. Required for the formation of functional ARID3A DNA-binding complexes.
COFACTOR: Binds 1 zinc ion per subunit.
SIZE: 659 amino acids; 76281 Da
SUBUNIT: Binds GTF2I through the PH domain. Interacts with SH3BP5 via the SH3 domain. Interacts with IBTK via its PH domain. Interacts with GTF2I and ARID3A.
SUBCELLULAR LOCATION: Cytoplasm (By similarity). Membrane; Peripheral membrane protein (By similarity). Nucleus (By similarity).
PTM: Autophosphorylated on Tyr-223 and Tyr-551. Phosphorylation of Tyr-223 may create a docking site for a SH2 containing protein (By similarity).
DISEASE: SwissProt: Q06187 # Defects in BTK are the cause of X-linked agammaglobulinemia type 1 (XLA) [MIM:300300]. 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. & Defects in BTK may be the cause of X-linked hypogammaglobulinemia and isolated growth hormone deficiency (XLA- IGHD) [MIM:307200]; also known as agammaglobulinemia and isolated growth hormone deficiency or Fleisher syndrome or isolated growth hormone deficiency type 3 (IGHD3). In rare cases XLA is inherited together with isolated growth hormone deficiency (IGHD).
SIMILARITY: SwissProt: Q06187 ## Belongs to the protein kinase superfamily. Tyr protein kinase family. TEC subfamily. & Contains 1 Btk-type zinc finger. & Contains 1 PH domain. & Contains 1 protein kinase domain. & Contains 1 SH2 domain. & Contains 1 SH3 domain.
COFACTOR: Binds 1 zinc ion per subunit.
SIZE: 659 amino acids; 76281 Da
SUBUNIT: Binds GTF2I through the PH domain. Interacts with SH3BP5 via the SH3 domain. Interacts with IBTK via its PH domain. Interacts with GTF2I and ARID3A.
SUBCELLULAR LOCATION: Cytoplasm (By similarity). Membrane; Peripheral membrane protein (By similarity). Nucleus (By similarity).
PTM: Autophosphorylated on Tyr-223 and Tyr-551. Phosphorylation of Tyr-223 may create a docking site for a SH2 containing protein (By similarity).
DISEASE: SwissProt: Q06187 # Defects in BTK are the cause of X-linked agammaglobulinemia type 1 (XLA) [MIM:300300]. 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. & Defects in BTK may be the cause of X-linked hypogammaglobulinemia and isolated growth hormone deficiency (XLA- IGHD) [MIM:307200]; also known as agammaglobulinemia and isolated growth hormone deficiency or Fleisher syndrome or isolated growth hormone deficiency type 3 (IGHD3). In rare cases XLA is inherited together with isolated growth hormone deficiency (IGHD).
SIMILARITY: SwissProt: Q06187 ## Belongs to the protein kinase superfamily. Tyr protein kinase family. TEC subfamily. & Contains 1 Btk-type zinc finger. & Contains 1 PH domain. & Contains 1 protein kinase domain. & Contains 1 SH2 domain. & Contains 1 SH3 domain.
Species:
Human
Molecular Weight:
78.4kDa
Quality Assurance:
routinely evaluated by phosphorylation of Src substrate peptide
Brand Family:
Upstate
Source:
expressed by baculovirus in Sf21 insect cells
Protein Target:
BTK
UniProt Number:
Specific Activity:
For Specific Activity data, refer to the Certificate of Analysis for individual lots of this enzyme.
Target Sub-Family:
TK
Storage Conditions:
6 months at -70°C
Promotional Text:
This enzyme is part of KinaseProfiler™, a custom inhibitor selectivity discovery service.
Packaging:
Also available in 250 µg size --call for pricing and availability and reference catalog number 14-552M when ordering the 250 µg size.
Gene Symbol:
- BTK
- PSCTK1
- MGC126261
- ATK
- XLA
- IMD1
- AT
- OTTHUMP00000063593
- BPK
- MGC126262
- AGMX1
Protein or Enzyme Type:
- Kinases
- Signal Transduction
Product Name:
BTK, active
Entrez Gene Number:
Purification Method:
Ni2+/NTA-agarose