09-456
Anti-AIRE Antibody
Upstate®, from rabbit
Synonym(s):
APECED protein, Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy
protein, autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2
autoimmune regulator
, autoimmune regulator (APECED protein), autoimmune
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About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
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
Western blot: 1:1000
Detect AIRE using this Anti-AIRE Antibody validated for use in WB.
Research Category
Inflammation & Immunology
Inflammation & Immunology
Research Sub Category
Developmental Signaling
Transcription Factors
RNA Binding Protein (RBP)
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
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
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.
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Storage Class Code
10 - Combustible liquids
WGK
WGK 2
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
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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
Wenyu Jiang et al.
The Journal of experimental medicine, 202(6), 805-815 (2005-09-21)
Loss of function mutations in the autoimmune regulator (Aire) gene in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy patients and mutant mice lead to autoimmune manifestations that segregate as a monogenic trait, but with wide variation in the spectrum of organs targeted. To investigate
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
Rikard Holmdahl
European journal of immunology, 37(3), 598-601 (2007-02-27)
Positional cloning of the underlying genes for the rare syndrome autoimmune polyendocrinopathy candidadiasis extrodermal dystrophy (APECED) opened a new venue of research on the role of central tolerance in autoimmunity. The associated autoimmune regulator gene (AIRE), was found to be
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
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