AIRE-1 (phospho Ser156) rabbit pAb

AIRE-1 (phospho Ser156) rabbit pAb

AO-06-ES5771-100

AIRE-1 (phospho Ser156) rabbit pAb 100μL

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Antibody Product Overview

ELK.NoES5771
Product nameAIRE-1 (phospho Ser156) rabbit pAb
ReactivityHuman;Rat;Mouse;
ApplicationsWB;ELISA
Other nameAIRE; APECED; Autoimmune regulator; Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein; APECED protein
Size100μL
Unit price ($)248
Human gene ID326
Human Swiss-ProtO43918
SourceRabbit
IsotypeIgG
TargetAIRE-1
Fields>>Ubiquitin mediated proteolysis;>>Primary immunodeficiency
Gene nameAIRE
Protein nameAutoimmune regulator
Human gene linkView Human Gene
Human Swiss linkView Human Swiss-Prot
Mouse gene ID
Mouse gene link
Mouse Swiss-ProtQ9Z0E3
Mouse Swiss linkView Mouse Swiss-Prot
Rat gene ID
Rat gene link
Rat Swiss-Prot
Rat Swiss link
ImmunogenThe antiserum was produced against synthesized peptide derived from human AIRE around the phosphorylation site of Ser156. AA range:126-175
SpecificityPhospho-AIRE-1 (S156) Polyclonal Antibody detects endogenous levels of AIRE-1 protein only when phosphorylated at S156.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
ClonalityPolyclonal
DilutionWestern Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration1 mg/ml
Storage stability-20°C/1 year
Molecular Weight (Da)
Observed band (KD)50kD
BackgroundThis gene encodes a transcriptional regulator that forms nuclear bodies and interacts with the transcriptional coactivator CREB binding protein. The encoded protein plays an important role in immunity by regulating the expression of autoantigens and negative selection of autoreactive T-cells in the thymus. Mutations in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendocrinopathy with candidiasis and ectodermal dystrophy (APECED). [provided by RefSeq, Jun 2012],
Functionalternative products:Additional isoforms seem to exist. Experimental confirmation may be lacking for some isoforms,disease: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
Subcellular locationNucleus . Cytoplasm . Predominantly nuclear but also cytoplasmic (PubMed:11274163, PubMed:14974083). Found in nuclear body-like structures (dots) and in a filamentous vimentin-like pattern (PubMed:11274163, PubMed:14974083, PubMed:26084028). Associated with tubular structures (PubMed:11274163, PubMed:14974083). .
ExpressionWidely 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. In secondary lymphoid organs, expressed in a discrete population of bone marrow-derived toleregenic antigen presenting cells (APCs) called extrathymic AIRE expressing cells (eTAC)(at protein level) (PubMed:23993652). Isoform 2 and isoform 3 seem to be less frequently expressed than isoform 1, if at all.

Additional Images

Image 1
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Western blot analysis of lysates from HeLa cells treated with Hu 2nM 24h, using AIRE (Phospho-Ser156) Antibody. The lane on the right is blocked with the phospho peptide.
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: AO-06-ES5771-100
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