Anti-TGF beta Receptor I Antibody (RMAB-0251149)
Cat. No.: RMAB-0251149
Category: Primary Antibodies
INQUIRY
100 μL
Customer Size
Rabbit monoclonal to TGF beta Receptor I
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | EPR20923-13 |
| Form | Liquid |
| Purity | Protein A purified |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Synthetic peptide. |
| Applications | IP, WB |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to TGF beta Receptor I; Suitable for: IP, WB; Knockout validated; Reacts with: Mouse, Rat, Human |
Target Information
| Target Symbol | TGFBR1 |
|---|---|
| Target Name | TGF-beta receptor type-1 |
| UniProt ID | P36897 |
| Cellular Localization | Membrane. |
| Function | On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for TGF-beta. |
| Involvement in Disease | Defects in TGFBR1 are the cause of Loeys-Dietz syndrome type 1A (LDS1A); also known as Furlong syndrome or Loeys-Dietz aortic aneurysm syndrome (LDAS). LDS1 is an aortic aneurysm syndrome with widespread systemic involvement. The disorder is characterized by arterial tortuosity and aneurysms, craniosynostosis, hypertelorism, and bifid uvula or cleft palate. Other findings include exotropy, micrognathia and retrognathia, structural brain abnormalities, intellectual deficit, congenital heart disease, translucent skin, joint hyperlaxity and aneurysm with dissection throughout the arterial tree.Defects in TGFBR1 are the cause of Loeys-Dietz syndrome type 2A (LDS2A). LDS2 is an aortic aneurysm syndrome with widespread systemic involvement. Physical findings include prominent joint laxity, easy bruising, wide and atrophic scars, velvety and translucent skin with easily visible veins, spontaneous rupture of the spleen or bowel, diffuse arterial aneurysms and dissections, and catastrophic complications of pregnancy, including rupture of the gravid uterus and the arteries, either during pregnancy or in the immediate postpartum period. LDS2 is characterized by the absence of craniofacial abnormalities with the exception of bifid uvula that can be present in some patients.Defects in TGFBR1 are the cause of aortic aneurysm familial thoracic type 5 (AAT5). Aneurysms and dissections of the aorta usually result from degenerative changes in the aortic wall. Thoracic aortic aneurysms and dissections are primarily associated with a characteristic histologic appearance known as 'medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance. |
| Post-translational Modifications | Phosphorylated at basal levels in the absence of ligand binding. Activated by multiple phosphorylation, mainly in the GS region. |
| Sequence Similarities | Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily. Contains 1 GS domain. Contains 1 protein kinase domain. |
Storage & Shipping
| Storage Buffer | pH: 7.40; Preservative: 0.01% Sodium azide; Constituents: 59.94% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA |
|---|---|
| Storage & Shipping | Shipped at 4°C. Store at 4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle. |
For research use only. Not for clinical use.
