- Catalog number
- M00759-2
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- TGFBR2 (transforming growth factor, beta receptor II (70/80kDa) ), also known as TGF-beta receptor type-2, TGFR-2, TGF-beta type II receptor, Transforming growth factor-beta receptor type II (TGF-beta receptor type II, TbetaR-II), is a member of the Ser/Thr protein kinase family and the TGFB receptor subfamily. A TGFBR2 cDNA encodes a deduced 565-amino acid protein with a calculated molecular mass of approximately 60 kD in length. The encoded protein is a transmembrane protein that has a protein kinase domain, forms a heterodimeric complex with another receptor protein, and binds TGF-beta. This receptor/ligand complex phosphorylates proteins, which then enter the nucleus and regulate the transcription of a subset of genes related to cell proliferation. Mutations in this gene have been associated with Marfan syndrome, Loeys-Deitz aortic aneurysm syndrome, Osler-Weber-Rendu syndrome, and the development of various types of tumors. Alternatively spliced transcript variants encoding different informs have been characterized.
- Synonyms
- SRK; STD; TZK; ZAP70; ZAP-70; ZAP 70; P43403
- Gene Name
- TGFBR2
- Gene ID
- 7048
- UniProt
- P37173
- Host
- Mouse
- Reactivity
- Human
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- A synthetic peptide corresponding to a sequence at the N-terminus of human TGFBR2, different from the related mouse sequence by five amino acids, and from the related rat sequence by eight amino acids.
- Clonality
- Monoclonal
- Clone
- Clone: 2F11
- Tissue Specificity
- Expressed in T- and natural killer cells. Also present in early thymocytes and pro/pre B-cells.
- Applications
- WB, IHC, ICC, IF, Flow Cytometry
- Field of Research
- Diabetes-associated, Heart Disease, Metabolism, Protein Phosphorylation, Ser/Thr Kinases, Serine/Threonine Kinases, Signal Transduction
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Tyrosine kinase that plays an essential role in regulation of the adaptive immune response. Regulates motility, adhesion and cytokine expression of mature T-cells, as well as thymocyte development. Contributes also to the development and activation of primary B-lymphocytes. When antigen presenting cells (APC) activate T-cell receptor (TCR), a serie of phosphorylations lead to the recruitment of ZAP70 to the doubly phosphorylated TCR component CD247/CD3Z through ITAM motif at the plasma membrane. This recruitment serves to localization to the stimulated TCR and to relieve its autoinhibited conformation. Release of ZAP70 active conformation is further stabilized by phosphorylation mediated by LCK. Subsequently, ZAP70 phosphorylates at least 2 essential adapter proteins: LAT and LCP2. In turn, a large number of signaling molecules are recruited and ultimately lead to lymphokine production, T-cell proliferation and differentiation. Furthermore, ZAP70 controls cytoskeleton modifications, adhesion and mobility of T-lymphocytes, thus ensuring correct delivery of effectors to the APC. ZAP70 is also required for TCR-CD247/CD3Z internalization and degradation through interaction with the E3 ubiquitin-protein ligase CBL and adapter proteins SLA and SLA2. Thus, ZAP70 regulates both T-cell activation switch on and switch off by modulating TCR expression at the T-cell surface. During thymocyte development, ZAP70 promotes survival and cell-cycle progression of developing thymocytes before positive selection (when cells are still CD4/CD8 double negative) . Additionally, ZAP70-dependent signaling pathway may also contribute to primary B-cells formation and activation through B-cell receptor (BCR) .
- References & Citations
- 1. Hagen, G., Muller, S., Beato, M., Suske, G. Cloning by recognition site screening of two novel GT box binding proteins: a family of Sp1 related genes. Nucleic Acids Res. 20: 5519-5525, 1992. 2. Lin, H. Y., Wang, X.-F., Ng-Eaton, E., Weinberg, R. A., Lodish, H. F. Expression cloning of the TGF-beta type II receptor, a functional transmembrane serine/threonine kinase. Cell 68: 775-785, 1992. 3. Hahm, K.-B., Cho, K., Lee, C., Im, Y.-H., Chang, J., Choi, S.-G., Sorensen, P. H. B., Thiele, C. J., Kim, S.-J.Repression of the gene encoding the TGF-beta type II receptor is a major target of the EWS-FLI1 oncoprotein. Nature Genet. 23: 222-227, 1999.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Observed Molecular Weight
- 70-85 kDa
- Applications Notes
- 6
- Gene Name Synonym
- Zeta chain of T cell receptor associated protein kinase 70kDa
- Subcellular Location
- Cell membrane. Cytoplasm. Membrane.
- Protein Name
- Zeta chain of T cell receptor associated protein kinase 70
- Isotype
- Mouse IgG2b
- Contents
- Each vial contains 4mg Trehalose, 0.9mg NaCl and 0.2mg Na2HPO4.