干扰素-gamma受体2/干扰素-gamma受体β/IFN-γRβ抗体
规格：1mg/1ml
英文名： IFNGR2
别名： IFNGR2; IFN gamma Receptor beta; AF 1; AF1; IFGR 2; IFGR2; IFNGR 2; IFNGT 1; IFNGT1; Interferon gamma receptor 2; Interferon gamma receptor accessory factor 1; Interferon gamma receptor beta chain; In
分子量： 36kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human IFNGR2
交叉反应：Human, Mouse, Rat, Dog, Cow, Rabbit,
细胞定位：细胞膜
干扰素-gamma受体2/干扰素-gamma受体β/IFN-γRβ抗体产品介绍：background: IFN gamma receptor beta is part of the receptor for interferon gamma. This class II cytokine receptor pairs with CDw119 to form the IFN gamma receptor and is an integral part of the IFN gamma signal transduction pathway. CDw119 serves as the IFN gamma binding chain and associates with the IFN gamma beta chain which is required for receptor signaling. The extracellular portion of both the IFN gamma receptor alpha and beta chains must be species matched. The IFN gamma receptor beta chain is expressed on T and B cells, NK cells, monocytes/ macrophages, and fibroblasts. Binding of IFN gamma induces receptor dimerization, internalization, Jak1 and Jak2 protein kinase activation and, ultimately, STAT1 activation. It is also likely to interact with GAF. IFN gamma initiates and regulates a variety of immune responses and is required for signal transduction. Contains 2 fibronectin type III domains. Defects in IFN gamma Receptor beta are a cause of mendelian susceptibility to mycobacterial disease (MSMD), a rare condition that confers predisposition to illness caused by several mycobacteria strains. Function: Part of the receptor for interferon gamma. Required for signal transduction. This accessory factor is an integral part of the IFN-gamma signal transduction pathway and is likely to interact with GAF, JAK1, and/or JAK2. Subcellular Location: Membrane; Single-pass type I membrane protein. DISEASE: Defects in IFNGR2 are a cause of mendelian susceptibility to mycobacterial disease (MSMD) [MIM:209950]; 干扰素-gamma受体2/干扰素-gamma受体β/IFN-γRβ抗体also known as familial disseminated atypical mycobacterial infection. This rare condition confers predisposition to illness caused by moderately virulent mycobacterial species, such as Bacillus Calmette-Guerin (BCG) vaccine and environmental non-tuberculous mycobacteria, and by the more virulent Mycobacterium tuberculosis. Other microorganisms rarely cause severe clinical disease in individuals with susceptibility to mycobacterial infections, with the exception of Salmonella which infects less than 50% of these individuals. The pathogenic mechanism underlying MSMD is the impairment of interferon-gamma mediated immunity, whose severity determines the clinical outcome. Some patients 干扰素-gamma受体2/干扰素-gamma受体β/IFN-γRβ抗体die of overwhelming mycobacterial disease with lepromatous-like lesions in early childhood, whereas others develop, later in life, disseminated but curable infections with tuberculoid granulomas. MSMD is a genetically heterogeneous disease with autosomal recessive, autosomal dominant or X-linked inheritance. Similarity: Belongs to the type II cytokine receptor family. Contains 2 fibronectin type-III domains. Gene ID: 3460 Database links: Entrez Gene: 3460 Human Entrez Gene: 15980 Mouse Entrez Gene: 360697 Rat Omim: 147569 Human SwissProt: P38484 Human Unigene: 634632 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
干扰素-gamma受体2/干扰素-gamma受体β/IFN-γRβ抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 **学 转录调节因子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid