产品编号 | bs-19843R |
英文名称 | Rabbit Anti-RFXANK antibody |
中文名称 | 调节因子X相关锚蛋白RFXANK抗体 |
别 名 | ANKRA1; Ankyrin repeat containing regulatory factor X associated protein; Ankyrin repeat family A protein 1; BLS; DNA-binding protein RFXANK; F14150_1; MGC138628; Regulatory factor X associated ankyrin containing protein; Regulatory factor X subunit B; Regulatory factor X-associated ankyrin-containing protein; RFX-B; RFXANK; RFXB; RFXB delta 4; RFXK_HUMAN. |
研究领域 | 细胞生物 免疫学 结合蛋白 细胞类型标志物 淋巴细胞 t-淋巴细胞 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | (predicted: Human,Mouse,Rat,Pig,Sheep,Cow,Horse,Chimpanzee) |
产品应用 | ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
理论分子量 | 28kDa |
细胞定位 | 细胞核 |
性 状 | Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human RFXANK: 31-130/260 |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
产品介绍 |
Defects in RFXANK are a cause of bare lymphocyte syndrome type 2 (BLS2) [MIM:209920]; also known as hereditary MHC class II deficiency or HLA class II-deficient combined immunodeficiency. BLS2 is a severe combined immunodeficiency disease with early onset. It is characterized by a profound defect in constitutive and interferon-gamma induced MHC II expression, absence of cellular and humoral T-cell response to antigen challenge, hypogammaglobulinemia and impaired antibody production. The consequence include extreme susceptibility to viral, bacterial and fungal infections. Function: Activates transcription from class II MHC promoters. Activation requires the activity of the MHC class II transactivator (MHC2TA). May regulate other genes in the cell. RFX binds the X1 box of MHC-II promoters. Isoform RFX-B-delta5 is not involved in the positive regulation of MHC class II genes. Subunit: The RFX heterotetrameric complex consists of 2 molecules of RFX5 and one each of RFXAP and RFX-B/RFXANK; with each subunit representing a separate complementation group. RFX forms cooperative DNA binding complexes with X2BP and CBF/NF-Y. RFX associates with MHC2TA to form an active transcriptional complex. Subcellular Location: Nucleus. Tissue Specificity: Ubiquitous. DISEASE: Defects in RFXANK are a cause of bare lymphocyte syndrome type 2 (BLS2) [MIM:209920]; also known as hereditary MHC class II deficiency or HLA class II-deficient combined immunodeficiency. BLS2 is a severe combined immunodeficiency disease with early onset. It is characterized by a profound defect in constitutive and interferon-gamma induced MHC II expression, absence of cellular and humoral T-cell response to antigen challenge, hypogammaglobulinemia and impaired antibody production. The consequence include extreme susceptibility to viral, bacterial and fungal infections. Similarity: Contains 5 ANK repeats. SWISS: O14593 Gene ID: 8625 Database links: Entrez Gene: 8625 Human Omim: 603200 Human SwissProt: O14593 Human Unigene: 153629 Human Unigene: 296776 Human |
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |