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].
Applications:Suitable for use in Western Blot, ELISA
DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
For maximum recovery of product, centrifuge the original vial prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phophatase (AP).
Specificity：Human
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu
Immunogen：RFX5 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 236-266 amino acids from the Central region of human RFX5.
