RFX-B is the gene responsible for the bare lymphocyte syndrome (BLS) which is characterized by a lack of MHC class II transcription.
Subsequently, there is a lack of B and T cell responses to foreign antigens.
RFX-B is a MHC class II transcription factor that is defective in BLS.
Regulatory factor X5 (RFX5) and Class II Transactivator (CIITA) are two transcriptional activators identified in human patients with bare lymphocyte syndrome.
They bind to "X boxes" in class II MHC gene promoters.
X boxes are conserved among class II MHC genes in humans and mice.
The X box sequence is 5'-GTNRCC(N 0-3 )RGYAAC-3' where N is any nucleotide, R is a purine, and Y is a pyrimidine.
Patients with bare lymphocyte syndrome have no class I or class II MHC molecules due to a complete lack of transcription of class I and class II MHC genes.
Both class I and class II MHC genes are flanked by 5’ promoter sequences, which are bound by specific transcription factors.
Consequences of Bare Lymphocyte Syndrome are an absence of cellular and humoral T cell immuneresponses.
Applications:Western Blot: 1:250-1:500
Storage and Stability:May be stored at 4°C for short-term only.
For long-term storage, store at -20°C.
Aliquots are stable for at least 12 months at -20°C.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Further dilutions can be made in assay buffer.
仕様
Size:50ug
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by immunoaffinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.2, 0.01% sodium azide before the addition of 40% glycerol.
Specificity:Reacts with residues 18–31 [ASELGDPEDPGEEAC] of the RFX-B protein.
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu
Immunogen:Synthetic peptide, aa18–31 [ASELGDPEDPGEEAC] of the RFX-B protein.