Autoimmune Regulator (AIRE) Antibody
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Description
AIRE Antibody is a Rabbit Polyclonal antibody against AIRE. 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). [provided by RefSeq, Jun 2012].
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Autoimmune Regulator (AIRE) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | WB: 1/500 - 1/2000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 60 µl |
| Size 2 | 120 µl |
| Size 3 | 200 µl |
| Form | Liquid |
| Tested Applications | WB |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | O43918 |
| Gene ID | 326 |
| NCBI Accession | NP_000374.1 |
| Alias | AIRE1,APECED,APS1,APSI,PGA1 |
| Background | Antibody anti-AIRE |
| Status | RUO |
| Note | Concentration: 1 mg/ml - |
Descripción
The Autoimmune Regulator (AIRE) is a protein encoded by the AIRE gene, and it plays a critical role in immune tolerance and the prevention of autoimmune diseases. AIRE is primarily expressed in a specialized type of cell called medullary thymic epithelial cells (mTECs) found in the thymus gland. AIRE functions as a transcriptional regulator, controlling the expression of a wide range of tissue-specific antigens (TSAs) within mTECs. These TSAs represent proteins that are normally expressed in various tissues. By inducing the expression of TSAs in the thymus, AIRE ensures that developing T cells encounter a diverse array of self-antigens during their maturation process.The exposure of developing T cells to self-antigens in the thymus is crucial for their selection and education. T cells that recognize self-antigens too strongly or too weakly are eliminated or rendered tolerant, respectively, through a process known as central tolerance. AIRE-mediated expression of TSAs in mTECs is a key mechanism by which central tolerance is maintained, helping to prevent the development of autoimmune responses against self-tissues. Mutations in the AIRE gene lead to a rare autoimmune disorder known as autoimmune polyendocrine syndrome type 1 (APS-1), also called autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). APS-1 is characterized by the development of multiple autoimmune diseases affecting various endocrine organs, such as the adrenal glands, parathyroid glands, and pancreas, as well as other tissues. These autoimmune manifestations result from defects in central tolerance due to impaired AIRE function, leading to the escape of autoreactive T cells from thymic deletion.
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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.
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AIRE Antibody is a Rabbit Polyclonal antibody against AIRE. 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). [provided by RefSeq, Jun 2012].
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