Product nameAnti-AIRE antibody
See all AIRE primary antibodies
DescriptionRabbit polyclonal to AIRE
Specificityab65040 detects endogenous levels of total AIRE protein.
Tested applicationsSuitable for: ELISA, ICC/IF, WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse
- WB: 293 cell extracts. IF: HeLa cells.
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Storage bufferpH: 7.40
Preservative: 0.02% Sodium azide
Constituents: PBS, 50% Glycerol, 0.87% Sodium chloride
Without Mg2+ and Ca2+
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab65040 was affinity purified from rabbit antiserum by affinity chromatography using epitope specific immunogen.
Our Abpromise guarantee covers the use of ab65040 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||1/500 - 1/1000.|
|WB||1/500 - 1/1000. Detects a band of approximately 65 kDa (predicted molecular weight: 58 kDa).|
FunctionTranscriptional regulator that binds to DNA as a dimer or as a tetramer, but not as a monomer. Binds to G-doublets in an A/T-rich environment; the preferred motif is a tandem repeat of 5'-. ATTGGTTA-3' combined with a 5'-TTATTA-3' box. Binds to nucleosomes (By similarity). Binds to chromatin and interacts selectively with histone H3 that is not methylated at 'Lys-4', not phosphorylated at 'Thr-3' and not methylated at 'Arg-2'. Functions as a sensor of histone H3 modifications that are important for the epigenetic regulation of gene expression. Functions as a transcriptional activator and promotes the expression of otherwise tissue-specific self-antigens in the thymus, which is important for self tolerance and the avoidance of autoimmune reactions.
Tissue specificityWidely expressed. Expressed at higher level in thymus (medullary epithelial cells and monocyte-dendritic cells), pancreas, adrenal cortex and testis. Expressed at lower level in the spleen, fetal liver and lymph nodes. Isoform 2 and isoform 3 seem to be less frequently expressed than isoform 1, if at all.
Involvement in diseaseDefects in AIRE are a cause of autoimmune poly-endocrinopathy candidiasis ectodermal dystrophy (APECED) [MIM:240300]; also known as autoimmune polyglandular syndrome type I (APS-1). APECED is an autosomal recessive disease characterized by: (1) autoimmune polyendocrinopathies: hypoparathyroidism, adrenocortical failure, IDDM, gonadal failure, hypothyroidism, pernicious anemia, and hepatitis; (2) chronic mucocutaneous candidiasis; (3) ectodermal dystrophies: vitiligo, alopecia, keratopathy, dystrophy of dental enamel, nails and tympanic membranes. In addition, a high proportion of patients develop squamous cell carcinoma of the oral mucosa. The disease is reported worldwide but is exceptionally prevalent among the Finnish population (incidence 1:25000) and the Iranian jews (incidence 1:9000).
Note=Most of the mutations alter the nucleus-cytoplasm distribution of AIRE and disturb its association with nuclear dots and cytoplasmic filaments. Most of the mutations also decrease transactivation of the protein. The HSR domain is responsible for the homomultimerization activity of AIRE. All the missense mutations of the HSR and the SAND domains decrease this activity, but those in other domains do not. The AIRE protein is present in soluble high-molecular-weight complexes. Mutations in the HSR domain and deletion of PHD zinc fingers disturb the formation of these complexes.
Sequence similaritiesContains 1 HSR domain.
Contains 2 PHD-type zinc fingers.
Contains 1 SAND domain.
DomainThe L-X-X-L-L repeats may be implicated in binding to nuclear receptors.
The HSR domain is required for localization on tubular structures (N-terminal part) and for homodimerization.
Interacts via the first PHD domain with the N-terminus of histone H3 that is not methylated at 'Lys-4'. Disruption of the first PHD domain has been shown to lead to reduced transcriptional activity and to localization of the protein mainly in the cytoplasm in small granules. While the PHD zinc fingers are necessary for the transactivation capacity of the protein, other regions also modulate this function.
modificationsPhosphorylated. Phosphorylation could trigger oligomerization.
Cellular localizationNucleus. Cytoplasm. Associated with tubular structures and in discrete nuclear dots resembling ND10 nuclear bodies. May shuttle between nucleus and cytoplasm.
- Information by UniProt
- AIRE antibody
- AIRE_HUMAN antibody
- AIRE1 antibody
ab65040, at a 1/500 dilution, staining AIRE in HeLa cells by Immunofluorescence, in the absence or presence of the immunising peptide.
All lanes : Anti-AIRE antibody (ab65040) at 1/500 dilution
Lane 1 : 293 cell extract
Lane 2 : 293 cell extract with immunising peptide at 10 µg
Lysates/proteins at 5 µg per lane.
Predicted band size: 58 kDa
Observed band size: 65 kDa why is the actual band size different from the predicted?
This product has been referenced in:
- Cai CQ et al. Both polymorphic variable number of tandem repeats and autoimmune regulator modulate differential expression of insulin in human thymic epithelial cells. Diabetes 60:336-44 (2011). WB ; Human . Read more (PubMed: 20876716) »