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09-456 Anti-AIRE Antibody

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09-456
100 µg  
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      개요

      Replacement Information

      주요 사양표

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      MWBRbAffinity PurifiedPolyclonal Antibody
      Description
      Catalogue Number09-456
      Brand Family Upstate
      Trade Name
      • Upstate
      DescriptionAnti-AIRE Antibody
      Alternate Names
      • APECED protein
      • Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy
        protein
      • autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2
        autoimmune regulator
      • autoimmune regulator (APECED protein)
      • autoimmune regulator (autoimmune polyendocrinopathy candidiasis
        ectodermal dystrophy)
      Background InformationAIRE (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).
      References
      Product Information
      FormatAffinity Purified
      Control
      • Murine thymus tissue lysate
      Presentation100 μg of immunoaffinity purified rabbit polyclonal IgG in 173 μl of 0.02M PBS, 0.25M NaCl containing 0.1% sodium azide
      Quality LevelMQ100
      Applications
      ApplicationDetect AIRE using this Anti-AIRE Antibody validated for use in WB.
      Key Applications
      • Western Blotting
      Application NotesSuggested dilutions:
      Western blot: 1:1000
      Biological Information
      ImmunogenKLH-conjugated synthetic peptide corresponding to amino acids 162-181 of mouse AIRE
      Epitopea.a. 162-181
      HostRabbit
      SpecificityRecognizes murine AIRE, Mr 58 kDa (calculated). Appears at ~65 kDa in Western blots.
      IsotypeIgG
      Species Reactivity
      • Mouse
      Species Reactivity NoteMouse. Likely to cross-react with rat, based on sequence homology. Cross-reactivity with other species has not been tested
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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
      • AIRE
      • AIRE1
      • PGA1
      • APSI
      • APS1
      • PGAI
      • APECED
      Purification MethodImmunoAffinity Purified
      UniProt Number
      UniProt SummaryFUNCTION: 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 Weight65 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceRoutinely evaluated by Western Blot analysis.

      Western Blot:
      A 1:1000 dilution of this lot detected AIRE in murine thymus lysate
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage Conditions2 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 Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      카탈로그 번호 GTIN
      09-456 04053252332722

      Documentation

      Anti-AIRE Antibody MSDS

      타이틀

      물질안전보건자료(MSDS) 

      Anti-AIRE Antibody Certificates of Analysis

      TitleLot Number
      Anti-AIRE - 3537590 3537590
      Anti-AIRE - DAM1562536 DAM1562536
      Anti-AIRE -2603234 2603234
      Anti-AIRE -2632479 2632479
      Anti-AIRE Polyclonal Antibody 3016660

      References

      Reference overviewPub Med ID
      Aire-ing self antigen variability and tolerance.
      Holmdahl, Rikard
      Eur. J. Immunol., 37: 598-601 (2007)  2007

      17323409 17323409
      Development of autoimmunity against transcriptionally unrepressed target antigen in the thymus of Aire-deficient mice
      Kuroda, Noriyuki, et al
      J Immunol, 174:1862-70 (2005)  2005

      15699112 15699112
      The cellular mechanism of Aire control of T cell tolerance
      Anderson, Mark S, et al
      Immunity, 23:227-39 (2005)  2005

      16111640 16111640
      Modifier loci condition autoimmunity provoked by Aire deficiency
      Jiang, Wenyu, et al
      J Exp Med, 202:805-15 (2005)  2005

      16172259 16172259
      Gene dosage--limiting role of Aire in thymic expression, clonal deletion, and organ-specific autoimmunity.
      Liston, Adrian, et al.
      J. Exp. Med., 200: 1015-26 (2004)  2004

      요약 표시
      15492124 15492124
      Aire regulates negative selection of organ-specific T cells.
      Liston, Adrian, et al.
      Nat. Immunol., 4: 350-4 (2003)  2003

      12612579 12612579
      Aire deficient mice develop multiple features of APECED phenotype and show altered immune response.
      Ramsey, Chris, et al.
      Hum. Mol. Genet., 11: 397-409 (2002)  2002

      11854172 11854172
      Projection of an immunological self shadow within the thymus by the aire protein.
      Anderson, Mark S, et al.
      Science, 298: 1395-401 (2002)  2002

      요약 표시
      12376594 12376594

      Brochure

      Title
      RNA-Binding Protein Immunoprecipitation

      관련 제품 및 어플리케이션

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      카테고리

      Life Science Research > Antibodies and Assays > Primary Antibodies