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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| M | WB | Rb | Affinity Purified | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | 09-456 |
| Brand Family | Upstate |
| Trade Name |
|
| Description | Anti-AIRE Antibody |
| Alternate Names |
|
| Background Information | AIRE (Autoimmune Regulator) is a human gene that is expressed primarily in the thymus. It causes transcription of a wide selection of organ-specific genes, reducing the threat of autoimmunity occurring by allowing the elimination of autoreactive T cells via negative selection. Defects in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendo-crinopathy-candidiasis-ectodermal dystrophy (APECED). Disruption of AIRE results in the development of a range of autoimmune diseases, the most common clinical conditions in the syndrome are hypoparathyroidism, primary adrenocortical failure and chronic mucocutaneous candidiasis. Aire functions through initiating the transcription of a diverse set of self-antigens, such as insulin, in the thymus. This expression then allows maturing thymocytes to become tolerant towards peripheral organs, thereby suppressing autoimmune disease. Aire is atranscriptional regulator protein that binds to DNA as dimer and tetramer, but not as a monomer. It forms nuclear bodies and interacts with the transcriptional coactivator CBP (CREBBP). At least three splice variant mRNAs products have been described including one which results in a premature stop codon and a transcript predicted to be a candidate for nuclear-mediated decay (NMD). |
| Product Information | |
|---|---|
| Format | Affinity Purified |
| Control |
|
| Presentation | 100 μg of immunoaffinity purified rabbit polyclonal IgG in 173 μl of 0.02M PBS, 0.25M NaCl containing 0.1% sodium azide |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Detect AIRE using this Anti-AIRE Antibody validated for use in WB. |
| Key Applications |
|
| Application Notes | Suggested dilutions: Western blot: 1:1000 |
| Biological Information | |
|---|---|
| Immunogen | KLH-conjugated synthetic peptide corresponding to amino acids 162-181 of mouse AIRE |
| Epitope | a.a. 162-181 |
| Host | Rabbit |
| Specificity | Recognizes murine AIRE, Mr 58 kDa (calculated). Appears at ~65 kDa in Western blots. |
| Isotype | IgG |
| Species Reactivity |
|
| Species Reactivity Note | Mouse. Likely to cross-react with rat, based on sequence homology. Cross-reactivity with other species has not been tested |
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | This gene encodes a transcriptional regulator that forms nuclear bodies and interacts with the transcriptional coactivator CBP. At least three splice variant mRNAs products have been described including one which results in a premature stop codon and a transcript predicted to be a candidate for nuclear-mediated decay (NMD). Defects in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). |
| Gene Symbol |
|
| Purification Method | ImmunoAffinity Purified |
| UniProt Number |
|
| UniProt Summary | FUNCTION: SwissProt: O43918 # Probable transcriptional regulator protein that binds to DNA as dimer and 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. May be involved in immune regulation. SIZE: 545 amino acids; 57727 Da SUBUNIT: Homodimer and homotetramer. Interacts with CREBBP. SUBCELLULAR LOCATION: Nucleus. Cytoplasm. Note=Associated with tubular structures and in discrete nuclear dots resembling ND10 nuclear bodies. May shuttle between nucleus and cytoplasm. 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. Isoforms 2 and 3 seem to be less frequently expressed than isoform 1, if at all. DOMAIN: SwissProt: O43918 The 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. & 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. PTM: Phosphorylated. Phosphorylation could trigger oligomerization. DISEASE: SwissProt: O43918 # Defects 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). & 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. SIMILARITY: Contains 1 HSR domain. & Contains 2 PHD-type zinc fingers. & Contains 1 SAND domain. |
| Molecular Weight | 65 kDa |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Routinely evaluated by Western Blot analysis. Western Blot: A 1:1000 dilution of this lot detected AIRE in murine thymus lysate |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | 2 years at -20°C from date of shipment. Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance. Note: Variability in freezer temperatures below -20°C may cause glycerol-containing solutions to become frozen during storage. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |