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.
Applications:Suitable for use in Immunofluorescence, Western Blot and Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Immunofluorescence: 1:50-1:200Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.09% sodium azide, 50% glycerol.
Specificity：Recognizes human RFX5.
Isotype：IgG
Calc Applications Abbrev：IF IHC WB
Calc Crossreactivity：Hu
Immunogen：Recombinant protein corresponding to full length human RFX5.
