BD-PT7052

RFX5 Rabbit Polyclonal Antibody

常规抗体

RFX5

RFX5

WB

Human,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

5993

https://www.uniprot.org/uniprot/5993

P48382

https://www.uniprot.org/uniprotkb/P48382/entry

53970

https://www.uniprot.org/uniprot/53970

Q9JL61

https://www.uniprot.org/uniprotkb/Q9JL61

Synthesized peptide derived from human RFX5 AA range: 491-541

This antibody detects endogenous levels of RFX5 at Human/Mouse

WB 1:500-2000;IHC-p 1:50-300

68kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

Ubiquitous.

Nucleus.

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 MHC promoters. Recognizes X-boxes. Mediates cooperative binding between RFX and NF-Y. RFX binds the X1 box of MHC-II promoters.,online information:RFX5 mutation db,PTM:Phosphorylated.,similarity:Belongs to the RFX family.,subunit:RFX consists of at least three different subunits; RFXAP, RFX5 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 CIITA to form an active transcriptional complex.,tissue specificity:Ubiquitous.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.