Rabbit anti-Human RFX5, aa320-494 Polyclonal Antibody | anti-RFX5 antibody

RFX5, aa320-494 (Regulatory Factor X5)

Cat. Num. AAA618439

• Gene Names: RFX5; RFX5
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunoprecipitation
• Purity: Purified; Purified Ig
• Synonyms: RFX5; aa320-494; Polyclonal Antibody; aa320-494 (Regulatory Factor X5); Anti -RFX5; anti-RFX5 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human RFX5 aa 320 to 494. By Western blot it reacts with a major band at 75kD. May react with minor bands at 65 and 80kD.
• Purity/Purification: Purified; Purified Ig
• Form/Format: Supplied as a sterile filtered liquid in 0.01M Sodium Phosphate buffer, pH 7.4 containing 0.15 M Sodium Chloride with 0.01% sodium azide.

• Applicable Applications for anti-RFX5 antibody: ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP)
• Application Notes: Western blot: 1:1,000 using fibroblast and B-cell lysate. Immunoprecipitation EIA Supershift assay: 0.5-1.0ul per assay.
• Immunogen: RFX5 peptide corresponding to the region at amino acids 320 to 494 of human protein conjugated to KLH
• Preparation and Storage: -20 degree C

Related Product Information for anti-RFX5 antibody

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 immune

Product Categories/Family for anti-RFX5 antibody

Antibodies; Abs to Transcription Factors

NCBI and Uniprot Product Information

• NCBI GI #: 150439365
• NCBI GeneID: 5993
• UniProt Accession #: A6PVW7; Q5VWC3; P48382; Q53EW3; Q9UG77
• Molecular Weight: 11,739 Da
• NCBI Official Full Name: regulatory factor X, 5 (influences HLA class II expression)
• NCBI Official Synonym Full Names: regulatory factor X, 5 (influences HLA class II expression)
• NCBI Official Symbol: RFX5
• NCBI Official Synonym Symbols: RFX5
• NCBI Protein Information: DNA-binding protein RFX5; OTTHUMP00000014465; OTTHUMP00000081737; OTTHUMP00000082795; OTTHUMP00000082796; OTTHUMP00000196318; OTTHUMP00000196321; regulatory factor X 5
• UniProt Protein Name: Regulatory factor X, 5 (Influences HLA class II expression)
• Protein Family: DNA-binding protein
• UniProt Gene Name: RFX5
• UniProt Entry Name: A6PVW7_HUMAN

NCBI Description

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]

Uniprot Description

RFX5: Activates transcription from class II MHC promoters. Recognizes X-boxes. Mediates cooperative binding between RFX and NF-Y. RFX binds the X1 box of MHC-II promoters. Defects in RFX5 are a cause of bare lymphocyte syndrome type 2 (BLS2); also known as hereditary MHC class II deficiency or HLA class II-deficient combined immunodeficiency. BLS2 is a severe combined immunodeficiency disease with early onset. It is characterized by a profound defect in constitutive and interferon-gamma induced MHC II expression, absence of cellular and humoral T-cell response to antigen challenge, hypogammaglobulinemia and impaired antibody production. The consequence include extreme susceptibility to viral, bacterial and fungal infections. Belongs to the RFX family.

Protein type: Transcription factor; DNA-binding

Chromosomal Location of Human Ortholog: 1q21

Cellular Component: nucleus

Molecular Function: protein binding; DNA binding; transcription factor activity

Biological Process: regulation of transcription from RNA polymerase II promoter; transcription, DNA-dependent; negative regulation of transcription from RNA polymerase II promoter

Disease: Bare Lymphocyte Syndrome, Type Ii