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Merck
CN
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文件

09-456

Sigma-Aldrich

Anti-AIRE Antibody

Upstate®, from rabbit

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别名:
APECED protein, Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

protein, autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2

autoimmune regulator

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

生物来源

rabbit

质量水平

抗体形式

affinity purified immunoglobulin

抗体产品类型

primary antibodies

克隆

polyclonal

纯化方式

affinity chromatography

种属反应性

mouse

制造商/商品名称

Upstate®

技术

western blot: suitable

同位素/亚型

IgG

UniProt登记号

运输

wet ice

靶向翻译后修饰

unmodified

基因信息

mouse ... Aire(11634)

一般描述

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).

特异性

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.

免疫原

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

应用

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)

质量

Routinely evaluated by Western Blot analysis.

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

目标描述

65 kDa

外形

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

储存及稳定性

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.

分析说明

Control
Murine thymus tissue lysate

法律信息

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

免责声明

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

闪点(°F)

Not applicable

闪点(°C)

Not applicable


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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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