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Regulatory factor X, 5

RFX5
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] (from NCBI)
Top mentioned proteins: MHC, RFX, CIITA, RFXAP, BLS
Papers using RFX5 antibodies
Chlamydia pneumoniae directly interferes with HIF-1alpha stabilization in human host cells.
Supplier
Roy Craig R., In PLoS Pathogens, 2006
... goat anti-mouse-HRP and goat anti-rabbit-HRP (both from Jackson ImmunoResearch Laboratories); mouse anti-caspase-3 (BD Biosciences); rabbit anti-RFX5 (Rockland Immunochemicals); Alexa Fluor 488 ...
Papers on RFX5
Kaposi's Sarcoma-Associated Herpesvirus Latency-Associated Nuclear Antigen Inhibits Major Histocompatibility Complex Class II Expression by Disrupting Enhanceosome Assembly through Binding with the Regulatory Factor X Complex.
New
Verma et al., Reno, United States. In J Virol, May 2015
Our data show that LANA is capable of binding to all three components of the RFX complex, RFX-associated protein (RFXAP), RFX5, and RFX-associated ankyrin-containing protein (RFXANK), in vivo but binds more strongly with the RFXAP component in in vitro binding assays.
Ankyrin repeats of ANKRA2 recognize a PxLPxL motif on the 3M syndrome protein CCDC8.
New
Yang et al., Montréal, Canada. In Structure, May 2015
We have recently demonstrated that ankyrin repeats of ANKRA2 and the paralogous bare lymphocyte syndrome transcription factor RFXANK recognize PxLPxL/I motifs shared by megalin, three histone deacetylases, and RFX5.
Association of high CD4-positive T cell infiltration with mutations in HLA class II-regulatory genes in microsatellite-unstable colorectal cancer.
New
Kloor et al., Heidelberg, Germany. In Cancer Immunol Immunother, Mar 2015
Mutations in RFX5, CIITA, and RFXAP were found in 13 (28.9%), 3 (6.7%),
NLRC5 exclusively transactivates MHC class I and related genes through a distinctive SXY module.
New
Guarda et al., Lausanne, Switzerland. In Plos Genet, Mar 2015
ChIP-sequencing performed with Rfx5(-/-) cells, which lack the pivotal enhanceosome factor RFX5, demonstrated its strict requirement for NLRC5 recruitment.
MHC class I and II deficiencies.
Review
Etzioni et al., Haifa, Israel. In J Allergy Clin Immunol, 2014
These transacting factors are the class II transactivator and 3 subunits of regulatory factor X (RFX): RFX containing ankyrin repeats (RFXANK), the fifth member of the RFX family (RFX5), and RFX-associated protein (RFXAP).
Role of chromatin and transcriptional co-regulators in mediating p63-genome interactions in keratinocytes.
Buck et al., Buffalo, United States. In Bmc Genomics, 2013
Furthermore, we use a comprehensive in silico approach that leverages ENCODE data to identify several known TFs such as AP1, AP2 and novel TFs (RFX5 for e.g.) that can potentially cooperate with p63 to modulate its myriad biological functions in keratinocytes.
Genomic mapping of the MHC transactivator CIITA using an integrated ChIP-seq and genetical genomics approach.
Knight et al., Oxford, United Kingdom. In Genome Biol, 2013
Overall, we map 843 CIITA binding intervals involving 442 genes and find 95% of intervals are located outside the MHC and 60% not associated with RFX5 binding.
Regulation of gene expression in HBV- and HCV-related hepatocellular carcinoma: integrated GWRS and GWGS analyses.
Lu et al., China. In Int J Clin Exp Med, 2013
We obtained two common genes FCN2 and CXCL14, and two common transcription factors RFX5 and EZH2.
Solution structure of the heterotrimeric complex between the interaction domains of RFX5 and RFXAP from the RFX gene regulatory complex.
GeneRIF
Garvie et al., Baltimore, United States. In J Mol Biol, 2010
RFXAP(C) consists of two alpha-helices that form a V-shaped structure that packs within the RFX5(N)(2) staple.
HDAC2 deacetylates class II transactivator and suppresses its activity in macrophages and smooth muscle cells.
GeneRIF
Xu et al., Nanjing, China. In J Mol Cell Cardiol, 2009
TSA treatment enhances the association of CIITA with the transcription factor RFX5, which ameliorates the down-regulation of CIITA recruitment to target promoters by HDAC2.
Assembly of the RFX complex on the MHCII promoter: role of RFXAP and RFXB in relieving autoinhibition of RFX5.
GeneRIF
Boss et al., Baltimore, United States. In Biochim Biophys Acta, 2008
RFXAP and RFXB have roles in relieving autoinhibition of RFX5
Peroxisome proliferator-activated receptor gamma interacts with CIITA x RFX5 complex to repress type I collagen gene expression.
GeneRIF
Smith et al., Boston, United States. In J Biol Chem, 2007
PPARgamma is within the RFX5.CIITA complex as judged by co-immunoprecipitation and DNA affinity precipitation studies
Regulatory factor for X-box family proteins differentially interact with histone deacetylases to repress collagen alpha2(I) gene (COL1A2) expression.
GeneRIF
Smith et al., Boston, United States. In J Biol Chem, 2006
results demonstrate that RFX1 and RFX5 differentially interact with class I HDACs underlying the different pathways when repressing collagen synthesis
Mutation in a winged-helix DNA-binding motif causes atypical bare lymphocyte syndrome.
Impact
GeneRIF
Peterlin et al., San Francisco, United States. In Nat Immunol, 2002
The mutation for the fifth Bbare lymphocyte syndrome complementation group was found in RFX5 and was mapped to one of the arginines in a DNA-binding surface of this protein. Its wild-type counterpart restored function.
The bare lymphocyte syndrome and the regulation of MHC expression.
Review
Impact
Mach et al., Genève, Switzerland. In Annu Rev Immunol, 2000
The affected genes encode RFXANK, RFX5, RFXAP, and CIITA, four regulatory factors that are highly specific and essential for MHCII genes.
Structure of the winged-helix protein hRFX1 reveals a new mode of DNA binding.
Impact
Burley et al., New York City, United States. In Nature, 2000
DNA-binding defects in the protein RFX5 cause bare lymphocyte syndrome or major histocompatibility antigen class II deficiency.
Molecular genetics of the Bare lymphocyte syndrome.
Review
Reith et al., Genève, Switzerland. In Rev Immunogenet, 1999
These genes encode CIITA, RFXANK, RFX5 and RFXAP.
A gene encoding a novel RFX-associated transactivator is mutated in the majority of MHC class II deficiency patients.
Impact
Reith et al., Genève, Switzerland. In Nat Genet, 1998
The factors defective in groups A (CIITA), C (RFX5) and D (RFXAP) have been identified.
The RFX complex is crucial for the constitutive and CIITA-mediated transactivation of MHC class I and beta2-microglobulin genes.
Impact
van den Elsen et al., Leiden, Netherlands. In Immunity, 1998
Using type III BLS cell lines, we demonstrate that the RFX subunits RFX5 and RFXAP are crucial for constitutive and CIITA-induced MHC class I and beta2m transactivation.
Regulation of MHC class II genes: lessons from a disease.
Review
Impact
Reith et al., Genève, Switzerland. In Annu Rev Immunol, 1995
A genetic complementation cloning approach has led to the isolation of CIITA and RFX5, two essential MHC-II gene transactivators.
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