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09-456 Sigma-AldrichAnti-AIRE Antibody

Detect AIRE using this Anti-AIRE Antibody validated for use in WB.

Anti-AIRE Antibody : FDS (Fiches de données de sécurité), certificats d’analyse (CoA) et de qualité (CoQ), dossiers, brochures et autres documents disponibles.

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CoA
Références bibliographiques
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Research Category: Inflammation & Immunology Research Sub-Category: Developmental Signaling, Transcription Factors, RNA Binding Protein (RBP) Gene Symbol: AIRE, AIRE1, PGA1, APSI, APS1, PGAI, APECED UniProt Number: O43918

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Tableau de caractéristiques principal

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	Upstate
Description	Anti-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 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).

References

Product Information
Format	Affinity Purified
Control	Murine thymus tissue lysate
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	Western Blotting
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	Mouse
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	NM_000383.2 NM_000658.1
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	AIRE AIRE1 PGA1 APSI APS1 PGAI APECED
Purification Method	ImmunoAffinity Purified
UniProt Number	O43918
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

Physicochemical Information

Dimensions

Materials Information

Toxicological Information

Safety Information according to GHS

Safety Information

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	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 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.

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

Transport Information

Supplemental Information

Specifications

Global Trade Item Number
Référence	GTIN
09-456	04053252332722

Documentation

Anti-AIRE Antibody FDS

Titre
Fiche de données de sécurité des matériaux (FDS)

Anti-AIRE Antibody Certificats d'analyse

Titre	Numéro de lot
Anti-AIRE - 3537590	3537590
Anti-AIRE - DAM1562536	DAM1562536
Anti-AIRE -2603234	2603234
Anti-AIRE -2632479	2632479
Anti-AIRE Polyclonal Antibody	3016660

Références bibliographiques

Aperçu de la référence bibliographique	Nº PubMed
Aire-ing self antigen variability and tolerance. Holmdahl, Rikard Eur. J. Immunol., 37: 598-601 (2007) 2007	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) 2004	15699112
The cellular mechanism of Aire control of T cell tolerance Anderson, Mark S, et al Immunity, 23:227-39 (2005) 2004	16111640
Modifier loci condition autoimmunity provoked by Aire deficiency Jiang, Wenyu, et al J Exp Med, 202:805-15 (2005) 2004	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 Afficher le résumé Inactivation of the autoimmune regulator (Aire) gene causes a rare recessive disorder, autoimmune polyendocrine syndrome 1 (APS1), but it is not known if Aire-dependent tolerance mechanisms are susceptible to the quantitative genetic changes thought to underlie more common autoimmune diseases. In mice with a targeted mutation, complete loss of Aire abolished expression of an insulin promoter transgene in thymic epithelium, but had no effect in pancreatic islets or the testes. Loss of one copy of Aire diminished thymic expression of the endogenous insulin gene and the transgene, resulting in a 300% increase in islet-reactive CD4 T cells escaping thymic deletion in T cell receptor transgenic mice, and dramatically increased progression to diabetes. Thymic deletion induced by antigen under control of the thyroglobulin promoter was abolished in Aire homozygotes and less efficient in heterozygotes, providing an explanation for thyroid autoimmunity in APS1. In contrast, Aire deficiency had no effect on thymic deletion to antigen controlled by a systemic H-2K promoter. The sensitivity of Aire-dependent thymic deletion to small reductions in function makes this pathway a prime candidate for more subtle autoimmune quantitative trait loci, and suggests that methods to increase Aire activity would be a potent strategy to lower the incidence of organ-specific autoimmunity.	15492124
Aire regulates negative selection of organ-specific T cells. Liston, Adrian, et al. Nat. Immunol., 4: 350-4 (2003) 2003	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
Projection of an immunological self shadow within the thymus by the aire protein. Anderson, Mark S, et al. Science, 298: 1395-401 (2002) 2002 Afficher le résumé Humans expressing a defective form of the transcription factor AIRE (autoimmune regulator) develop multiorgan autoimmune disease. We used aire- deficient mice to test the hypothesis that this transcription factor regulates autoimmunity by promoting the ectopic expression of peripheral tissue- restricted antigens in medullary epithelial cells of the thymus. This hypothesis proved correct. The mutant animals exhibited a defined profile of autoimmune diseases that depended on the absence of aire in stromal cells of the thymus. Aire-deficient thymic medullary epithelial cells showed a specific reduction in ectopic transcription of genes encoding peripheral antigens. These findings highlight the importance of thymically imposed "central" tolerance in controlling autoimmunity.	12376594

Brochure

Titre
RNA-Binding Protein Immunoprecipitation

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Catégories

Life Science Research > Antibodies and Assays > Primary Antibodies

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