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Products Polyclonal Anti-TGFBR1
| Catalogue number: | PA1731 |
| Price: | $200.00 |
| Reactivities: | Human, Mouse, Rat |
| Applications: | Immunohistochemistry, Western Blot |
| Size: | 100ug/vial |
| Gene: | TGFBR1 |
| Swiss prot: | P36897 |
| Form: | Lyophilized |
| Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
| Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing. |
| Scientific background: | TGFBR1, Transforming growth factor, beta receptor I is a TGF beta receptor. TGFBR1 is its human gene. The protein encoded by this gene forms a heteromeric complex with type II TGF-beta receptors when bound to TGF-beta, transducing the TGF-beta signal from the cell surface to the cytoplasm. The encoded protein is a serine/threonine protein kinase. Mutations in this gene have been associated with Loeys-Dietz aortic aneurysm syndrome (LDAS). TGFB1 regulates cell cycle progression by a unique signaling mechanism that involves its binding to TGFBR2 and activation of TGFBR1. Both are transmembrane serine/threonine receptor kinases. The TGFBR1 receptor may be inactivated in many of the cases of human tumor cells refractory to TGFB-mediated cell cycle arrest. Vellucci and Reiss (1997) reported that the TGFBR1 gene is approximately 31 kb long and contains 9 exons. The organization of the segment of the gene that encodes the C-terminal portion of the serine/threonine kinase domain appears to be highly conserved among members of the gene family. |
| References: | 1.Drera, B., Tadini, G., Barlati, S., Colombi, M.Identification of a novel TGFBR1 mutation in a Loeys-Dietz syndrome type II patient with vascular Ehlers-Danlos syndrome phenotype. (Letter)Clin. Genet. 73: 290-293, 2008. 2.Goudie, D. R., D'Alessandro, M., Merriman, B., Lee, H., Szeverenyi, I., Avery, S., O'Connor, B. D., Nelson, S. F., Coats, S. E., Stewart, A., Christie, L., Pichert, G., and 11 others.Multiple self-healing squamous epithelioma is caused by a disease-specific spectrum of mutations in TGFBR1.Nature Genet. 43: 365-369, 2011. 3.Vellucci, V. F., Reiss, M.Cloning and genomic organization of the human transforming growth factor-beta type I receptor gene.Genomics 46: 278-283, 1997. |
| Additional info: | A synthetic peptide corresponding to a sequence in the middle region of human TGFBR1, identical to the related rat and mouse sequences. |
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