RFX5, ID (RFX5, DNA-binding protein RFX5, Regulatory factor X 5)

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].\n\nApplications:\nSuitable for use in Western Blot, ELISA\n\nRecommended Dilution:\nELISA: 1:1,000\nWestern Blot: 1:100-500\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

040988

Size

200ul

Applications

ELISA, WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Laird, K.M., et al. J. Mol. Biol. 403(1):40-51(2010)\nKong, X., et al. J. Mol. Cell. Cardiol. 46(3):292-299(2009)\nGarvie, C.W., et al. Biochim. Biophys. Acta 1779(12):797-804(2008)\nEnnis, S., et al. Lancet 372(9652):1828-1834(2008)\nXu, Y., et al. J. Biol. Chem. 282(36):26046-26056(2007)

Additional Info

Human

Alternative Names

RFX5, DNA-binding protein RFX5, Regulatory factor X 5