- Catalog number
- PB9980
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- The autoimmune regulator (AIRE) is a protein that in humans is encoded by the AIRE gene. This gene encodes a transcriptional regulator that forms nuclear bodies and interacts with the transcriptional coactivator CREB binding protein. The encoded protein plays an important role in immunity by regulating the expression of autoantigens and negative selection of autoreactive T-cells in the thymus. Mutations in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendocrinopathy with candidiasis and ectodermal dystrophy (APECED) .
- Synonyms
- Autoimmune regulator; Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein; APECED protein; AIRE; APECED
- Gene Name
- AIRE
- Gene ID
- 326
- UniProt
- O43918
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- E.coli-derived human AIRE recombinant protein (Position: M1-K120) . Human AIRE shares 87.2% amino acid (aa) sequence identity with mouse AIRE.
- Clonality
- Polyclonal
- Tissue Specificity
- Widely 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.
- Applications
- Flow Cytometry, IF, IHC, ICC, WB
- Field of Research
- Autoimmune,2339, Domain Families, Epigenetics and Nuclear Signaling, Immune System Diseases, Immunology, Transcription, Zinc Finger
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Transcriptional 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. .
- References & Citations
- 1. Lindmark E, Chen Y, Georgoudaki AM, Dudziak D, Lindh E, Adams WC, Loré K, Winqvist O, Chambers BJ, Karlsson MC, et al. (2013) . AIRE expressing marginal zone dendritic cells balances adaptive immunity and T-follicular helper cell recruitment.. Journal of Autoimmunity 42: 62–70. 2. Liston A, Lesage S, Wilson J, Peltonen L, Goodnow CC (April 2003) . Aire regulates negative selection of organ-specific T cells. Nat. Immunol. 4 (4) : 350–4.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 57727 MW
- Observed Molecular Weight
- 58-68 kDa
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- 6
- Gene Name Synonym
- Autoimmune regulator
- Subcellular Location
- Nucleus. Cytoplasm. Associated with tubular structures and in discrete nuclear dots resembling ND10 nuclear bodies. May shuttle between nucleus and cytoplasm.
- Protein Name
- Autoimmune regulator
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.