RFX5 rabbit pAb
- Catalog No.:YT7052
- Applications:WB;IHC
- Reactivity:Human;Mouse
- Target:
- RFX5
- Fields:
- >>Antigen processing and presentation;>>Tuberculosis;>>Primary immunodeficiency
- Gene Name:
- RFX5
- Protein Name:
- RFX5
- Human Gene Id:
- 5993
- Human Swiss Prot No:
- P48382
- Mouse Gene Id:
- 53970
- Mouse Swiss Prot No:
- Q9JL61
- 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.
- Source:
- Polyclonal, Rabbit,IgG
- Dilution:
- WB 1:500-2000;IHC 1:50-300
- Purification:
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- Concentration:
- 1 mg/ml
- Storage Stability:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- Molecular Weight(Da):
- 68kD
- 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.
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- Antibody-FAQs
- Products Images
- Western blot analysis of lysates from A431 cells, primary antibody was diluted at 1:1000, 4°over night
- 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).