免疫原
Synthesized peptide derived from human TGF β Receptor II
特异性
This antibody detects endogenous levels of human TGF β Receptor II. Heat-induced epitope retrieval (HIER) Citrate buffer of pH6.0 was highly recommended as antigen repair method in paraffin section
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Mouse, Monoclonal/IgG1, Kappa
稀释比例
IHC-p 1:100-500. IF 1:50-200
纯化工艺
The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
背景
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. [provided by RefSeq, Jul 2008],
功能
catalytic activity:ATP + [receptor-protein] = ADP + [receptor-protein] phosphate.,cofactor:Magnesium or manganese.,disease:Defects in TGFBR2 are a cause of esophageal cancer [MIM:133239].,disease:Defects in TGFBR2 are the cause of aortic aneurysm familial thoracic type 3 (AAT3) [MIM:610380]. 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.,disease:Defects in TGFBR2 are the cause of hereditary non-polyposis colorectal cancer type 6 (HN
