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Sigma-Aldrich

Anti-AIRE Antibody

Upstate®, from rabbit

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Synonym(s):
APECED protein, Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

protein, autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2

autoimmune regulator

, autoimmune regulator (APECED protein), autoimmune
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41

biological source

rabbit

Quality Level

antibody form

affinity purified immunoglobulin

antibody product type

primary antibodies

clone

polyclonal

purified by

affinity chromatography

species reactivity

mouse

manufacturer/tradename

Upstate®

technique(s)

western blot: suitable

isotype

IgG

NCBI accession no.

UniProt accession no.

shipped in

wet ice

target post-translational modification

unmodified

Gene Information

mouse ... Aire(11634)

General description

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).

Specificity

Likely to cross-react with rat, based on sequence homology. Cross-reactivity with other species has not been tested
Recognizes murine AIRE, Mr 58 kDa (calculated). Appears at ~65 kDa in Western blots.

Immunogen

Epitope: a.a. 162-181
KLH-conjugated synthetic peptide corresponding to amino acids 162-181 of mouse AIRE

Application

Suggested dilutions:
Western blot: 1:1000
Detect AIRE using this Anti-AIRE Antibody validated for use in WB.
Research Category
Inflammation & Immunology
Research Sub Category
Developmental Signaling

Transcription Factors

RNA Binding Protein (RBP)

Quality

Routinely evaluated by Western Blot analysis.

Western Blot:
A 1:1000 dilution of this lot detected AIRE in murine thymus lysate

Target description

65 kDa

Physical form

100 μg of immunoaffinity purified rabbit polyclonal IgG in 173 μl of 0.02M PBS, 0.25M NaCl containing 0.1% sodium azide
ImmunoAffinity Purified

Storage and Stability

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.

Analysis Note

Control
Murine thymus tissue lysate

Legal Information

UPSTATE is a registered trademark of Merck KGaA, Darmstadt, Germany

Disclaimer

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.

WGK

WGK 2

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


Certificates of Analysis (COA)

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Noriyuki Kuroda et al.
Journal of immunology (Baltimore, Md. : 1950), 174(4), 1862-1870 (2005-02-09)
Autoimmune regulator (AIRE) gene mutation is responsible for the development of organ-specific autoimmune disease with monogenic autosomal recessive inheritance. Although Aire has been considered to regulate the elimination of autoreactive T cells through transcriptional control of tissue-specific Ags in thymic
Mark S Anderson et al.
Science (New York, N.Y.), 298(5597), 1395-1401 (2002-10-12)
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
Mark S Anderson et al.
Immunity, 23(2), 227-239 (2005-08-23)
Aire promotes the tolerization of thymocytes by inducing the expression of a battery of peripheral-tissue antigens in thymic medullary epithelial cells. We demonstrate that the cellular mechanism by which Aire exerts its tolerance-promoting function is not primarily positive selection of
Chris Ramsey et al.
Human molecular genetics, 11(4), 397-409 (2002-02-21)
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a monogenic autosomal recessive disease caused by mutations in the AIRE gene. Here we have produced knock-out mice for the Aire gene. The Aire-/- mice develop normally; however, autoimmune features of APECED in Aire-/- mice
Adrian Liston et al.
Nature immunology, 4(4), 350-354 (2003-03-04)
Autoimmune polyendocrinopathy syndrome type 1 is a recessive Mendelian disorder resulting from mutations in a novel gene, AIRE, and is characterized by a spectrum of organ-specific autoimmune diseases. It is not known what tolerance mechanisms are defective as a result

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