Product nameAnti-TGF beta Receptor I antibody, prediluted
See all TGF beta Receptor I primary antibodies
DescriptionRabbit polyclonal to TGF beta Receptor I, prediluted
Tested applicationsSuitable for: IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Sheep, Horse, Chicken, Cow, Pig, Xenopus laevis
Synthetic peptide corresponding to TGF beta Receptor I.
- Human placenta tissue A549 cell
Storage instructionsShipped at 4°C. Store at +4°C.
Storage bufferpH: 7.60
Preservative: 0.1% Sodium azide
Constituents: 1% BSA, 0.605% Tris
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab53647 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionOn 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.
Tissue specificityFound in all tissues examined, most abundant in placenta and least abundant in brain and heart.
Involvement in diseaseDefects in TGFBR1 are the cause of Loeys-Dietz syndrome type 1A (LDS1A) [MIM:609192]; 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) [MIM:608967]. 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) [MIM:608967]. 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.
Sequence similaritiesBelongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 GS domain.
Contains 1 protein kinase domain.
modificationsPhosphorylated at basal levels in the absence of ligand binding. Activated by multiple phosphorylation, mainly in the GS region.
- Information by UniProt
- AAT 5 antibody
- AAT5 antibody
- Activin A receptor type II like kinase 53kDa antibody
ab53647 has not yet been referenced specifically in any publications.