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品种反应性 主要应用 宿主 格式 抗体类型 R, H, M WB, IHC Rb Purified Polyclonal Antibody 描述 产品目录编号 06-227 品牌系列 Upstate
商名 - Upstate
描述 Anti-TGFβR Type II Antibody, NT 产品信息 格式 Purified 演示 0.1M Tris-glycine, pH 7.4, 0.15M NaCl, 0.05% sodium azide before the addition of glycerol to 30% 应用 应用 Detect TGFβR Type II using this Anti-TGFβR Type II Antibody, NT validated for use in IH, IP & WB. 主要应用 - Western Blotting
- Immunohistochemistry
生物信息 免疫原品种 peptide corresponds to the first 28 N-Terminal residues of the mature human TGFb receptor 宿主 Rabbit 特异性 p75 TGFb Type II receptors 同种型 IgG 品种反应性 RatHumanMouse 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_003242
Entrez基因汇总 This gene encodes a member of the Ser/Thr protein kinase family and the TGFB receptor subfamily. 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, and the development of various types of tumors. Alternatively spliced transcript variants encoding different isoforms have been characterized. 基因符号 - TGFbeta-RII
- TGFBR2
- AAT3
- TAAD2
- TbetaR-II
- TGFR-2
- RIIC
- MFS2
- HNPCC6
- FAA3
纯化方法 Protein G chromatography UniProt编号 - P37173
UniProt汇总 FUNCTION: SwissProt: P37173 # 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.
COFACTOR: Magnesium or manganese (By similarity).
SIZE: 567 amino acids; 64568 Da
SUBUNIT: Binds to DAXX.
SUBCELLULAR LOCATION: Membrane; Single-pass type I membrane protein.
PTM: Phosphorylated on a Ser/Thr residue in the cytoplasmic domain.
DISEASE: SwissProt: P37173 # Defects in TGFBR2 are the cause of hereditary non- polyposis colorectal cancer type 6 (HNPCC6) [MIM:190182]. Mutations in more than one gene locus can be involved alone or in combination in the production of the HNPCC phenotype (also called Lynch syndrome). Most families with clinically recognized HNPCC have mutations in either MLH1 or MSH2 genes. HNPCC is an autosomal, dominantly inherited disease associated with marked increase in cancer susceptibility. It is characterized by a familial predisposition to early onset colorectal carcinoma (CRC) and extra-colonic cancers of the gastrointestinal, urological and female reproductive tracts. HNPCC is reported to be the most common form of inherited colorectal cancer in the Western world, and accounts for 15% of all colon cancers. Cancers in HNPCC originate within benign neoplastic polyps termed adenomas. Clinically, HNPCC is often divided into two subgroups. Type I: hereditary predisposition to colorectal cancer, a young age of onset, and carcinoma observed in the proximal colon. Type II: patients have an increased risk for cancers in certain tissues such as the uterus, ovary, breast, stomach, small intestine, skin, and larynx in addition to the colon. Diagnosis of classical HNPCC is based on the Amsterdam criteria: 3 or more relatives affected by colorectal cancer, one a first degree relative of the other two; 2 or more generation affected; 1 or more colorectal cancers presenting before 50 years of age; exclusion of hereditary polyposis syndromes. The term suspected HNPCC or incomplete HNPCC can be used to describe families who do not or only partially fulfill the Amsterdam criteria, but in whom a genetic basis for colon cancer is strongly suspected. HNPCC6 is a type of colorectal cancer complying with the clinical criteria of HNPCC, except that the onset of cancer was beyond 50 years of age in all cases. & Defects in TGFBR2 are a cause of esophageal cancer [MIM:133239]. & Defects in TGFBR2 are the cause of Marfan syndrome type II (MFS2) [MIM:154705]. Marfan syndrome (MFS) is an extracellular matrix disorder with cardinal manifestations in the eye, skeleton and cardiovascular systems, and is associated with defects in the gene encoding fibrillin 1 (FBN1). A second type of the disorder is caused by mutations in a second locus MFS2. Identification of a 3p24.1 chromosomal breakpoint disrupting the gene TGFBR2 in a Japanese individual with Marfan syndrome led to consider TGFBR2 as the gene underlying association with MFS at the MSF2 locus. & Defects in TGFBR2 are a cause of Loeys-Dietz aortic aneurysm syndrome (LDAS) [MIM:609192]. LDAS is an aortic aneurysm syndrome characterized by widely spaced eyes (hypertelorism), bifid uvula and/or cleft palate, and generalized arterial tortuosity with ascending aortic aneurysm and aortic dissection. This syndrome shows autosomal dominant inheritance and variable clinical expression. Other findings in multiple systems include craniosynostosis, structural brain abnormalities, mental retardation, congenital heart disease and aneurysms with dissection throughout the arterial tree. & Defects in TGFBR2 are the cause of familial thoracic aortic aneurysm 3 (AAT3) [MIM:608967]; also known as familial thoracic aortic aneurysm and dissection (TAAD). 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' or 'Erdheim cystic medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance. AAT3 is an autosomal dominant disorder with reduced penetrance and variable expression.
SIMILARITY: SwissProt: P37173 ## Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily. & Contains 1 protein kinase domain.产品使用声明 质量保证 routinely evaluated by immunoblot on TF-1 cell lysate, WI-38 human diploid fibroblasts or on rat L6 cell lysate 使用声明 - 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.
储存和货运信息 存储条件 2 years at 2-8°C 包装信息 数量 400 µg
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