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Antibody Product Overview
| ELK.No | ES13409 |
| Product name | RFX5 rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB;IHC |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 5993 |
| Human Swiss-Prot | P48382 |
| Source | Rabbit |
| Isotype | IgG |
| Target | RFX5 |
| Fields | >>Antigen processing and presentation;>>Tuberculosis;>>Primary immunodeficiency |
| Gene name | RFX5 |
| Protein name | RFX5 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 53970 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q9JL61 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human RFX5 AA range: 491-541 |
| Specificity | This antibody detects endogenous levels of RFX5 at Human/Mouse |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000;IHC-p 1:50-300 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 68kD |
| Observed band (KD) | |
| Background | A lack of MHC-II expression results in a severe immunodeficiency syndrome called MHC-II deficiency, or the bare lymphocyte syndrome (BLS; MIM 209920). At least 4 complementation groups have been identified in B-cell lines established from patients with BLS. The molecular defects in complementation groups B, C, and D all lead to a deficiency in RFX, a nuclear protein complex that binds to the X box of MHC-II promoters. The lack of RFX binding activity in complementation group C results from mutations in the RFX5 gene encoding the 75-kD subunit of RFX (Steimle et al., 1995). RFX5 is the fifth member of the growing family of DNA-binding proteins sharing a novel and highly characteristic DNA-binding domain called the RFX motif. Multiple alternatively spliced transcript variants have been found but the full-length natures of only two have been determined. [provided by RefSeq, Jul 2008], |
| Function | disease:Defects in RFX5 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.,domain:The N-terminus is required for its association with RFXANK and RFXAP, for assembly of the RFX complex, and for binding of this complex to its X box target site in the MHC-II promoter. The C-terminus mediates cooperative binding between the RFX complex and NF-Y.,function:Activates transcription from class II |
| Subcellular location | Nucleus. |
| Expression | Ubiquitous. |
Additional Images
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Western blot analysis of lysates from A431 cells, primary antibody was diluted at 1:1000, 4°over night
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Immunohistochemical analysis of paraffin-embedded human liver cancer. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).
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: AO-06-ES13409-100
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