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 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.
      • 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).