概述

  • 产品名称Anti-AIRE抗体
    参阅全部 AIRE 一抗
  • 描述
    山羊多克隆抗体to AIRE
  • 经测试应用适用于: IHC-Fr, WB, ELISA, IHC-Pmore details
  • 种属反应性
    与反应: Rat, Dog, Human
    预测可用于: Mouse
  • 免疫原

    Synthetic peptide:

    C-KDVDLSQPRKGRKP

    , corresponding to internal sequence amino acids 102-115 of Human AIRE (NP_000374.1).

  • 阳性对照
    • Human thymus tissue.

性能

应用

Our Abpromise guarantee covers the use of ab78065 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-Fr 1/200.
WB Use a concentration of 0.5 - 1 µg/ml. Predicted molecular weight: 58 kDa.
ELISA 1/32000.
IHC-P Use a concentration of 2.5 µg/ml.

靶标

  • 功能Transcriptional regulator that binds to DNA as a dimer or as a 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. Binds to nucleosomes (By similarity). Binds to chromatin and interacts selectively with histone H3 that is not methylated at 'Lys-4', not phosphorylated at 'Thr-3' and not methylated at 'Arg-2'. Functions as a sensor of histone H3 modifications that are important for the epigenetic regulation of gene expression. Functions as a transcriptional activator and promotes the expression of otherwise tissue-specific self-antigens in the thymus, which is important for self tolerance and the avoidance of autoimmune reactions.
  • 组织特异性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. Isoform 2 and isoform 3 seem to be less frequently expressed than isoform 1, if at all.
  • 疾病相关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).
    Note=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.
  • 序列相似性Contains 1 HSR domain.
    Contains 2 PHD-type zinc fingers.
    Contains 1 SAND domain.
  • 结构域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.
    Interacts via the first PHD domain with the N-terminus of histone H3 that is not methylated at 'Lys-4'. 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.
  • 翻译后修饰Phosphorylated. Phosphorylation could trigger oligomerization.
  • 细胞定位Nucleus. Cytoplasm. Associated with tubular structures and in discrete nuclear dots resembling ND10 nuclear bodies. May shuttle between nucleus and cytoplasm.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AIRE antibody
    • AIRE_HUMAN antibody
    • AIRE1 antibody
    • APECED antibody
    • APECED protein antibody
    • APS1 antibody
    • APSI antibody
    • Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein antibody
    • Autoimmune regulator antibody
    • Autoimmune regulator protein antibody
    • PGA1 antibody
    see all

Anti-AIRE antibody 图像

  • ab78065 at 2.5µg/ml staining AIRE in human thymus by Immunohistochemistry using formalin-fixed, paraffin-embedded tissue.
  • ab78065 staining AIRE in Human tonsil tissue sections by Immunohistochemistry (IHC-Fr - frozen sections). Tissue was fixed with acetone. Samples were incubated with primary antibody (1/200 in PBS + 1% BSA) for 16 hours at 4°C. A Biotin-conjugated Pig anti-goat polyclonal (1/320) was used as the secondary antibody.

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Anti-AIRE antibody (ab78065)参考文献

This product has been referenced in:
  • Skogberg G  et al. Altered expression of autoimmune regulator in infant down syndrome thymus, a possible contributor to an autoimmune phenotype. J Immunol 193:2187-95 (2014). IHC-P ; Human . Read more (PubMed: 25038256) »
  • Peters JH  et al. Human secondary lymphoid organs typically contain polyclonally-activated proliferating regulatory T cells. Blood 122:2213-23 (2013). IHC . Read more (PubMed: 23950176) »

See all 2 Publications for this product

Product Wall

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Human Tissue sections (Tonsil)
Specification Tonsil
Fixative Acetone
Permeabilization No
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提交于 Nov 23 2010

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"