Rabbit anti-Human, Mouse AIRE Polyclonal Antibody | anti-AIRE antibody

AIRE, ID (AIRE, APECED, Autoimmune regulator, Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein)

Cat. Num. AAA6004910

• Gene Names: AIRE; APS1; APSI; PGA1; AIRE1; APECED
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot, Immunofluorescence
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: AIRE; Polyclonal Antibody; ID (AIRE; APECED; Autoimmune regulator; Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein); Anti -AIRE; anti-AIRE antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human, mouse
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-AIRE antibody: ELISA (EL/EIA), Western Blot (WB), Immunofluorescence (IF)
• Application Notes: Suitable for use in Western Blot, Immunofluorescence, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Immunofluorescence: 1:10-50
• Immunogen: AIRE antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 64-92 amino acids from the Central region of human AIRE.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(AIRE Antibody (Center) western blot analysis in mouse kidney tissue lysates (35ug/lane).This demonstrates the AIRE antibody detected the AIRE protein (arrow).)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of AIRE Antibody (Center) with 293 cell followed by Alexa Fluor® 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)

Related Product Information for anti-AIRE antibody

This gene encodes a transcriptional regulator that forms nuclear bodies and interacts with the transcriptional coactivator CBP. 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). Defects in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).

Product Categories/Family for anti-AIRE antibody

Antibodies; Abs to Autoimmune

NCBI and Uniprot Product Information

• NCBI GI #: 3392940
• NCBI GeneID: 326
• UniProt Accession #: O43918; O43922; O43932; O75745; B2RP50
• Molecular Weight: 57,727 Da
• NCBI Official Full Name: AIRE
• NCBI Official Synonym Full Names: autoimmune regulator
• NCBI Official Symbol: AIRE
• NCBI Official Synonym Symbols: APS1; APSI; PGA1; AIRE1; APECED
• NCBI Protein Information: autoimmune regulator; autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein
• UniProt Protein Name: Autoimmune regulator
• Protein Family: Autoimmune regulator
• UniProt Gene Name: AIRE
• UniProt Synonym Gene Names: APECED; APECED protein
• UniProt Entry Name: AIRE_HUMAN

NCBI Description

This 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]

Uniprot Description

AIRE: 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. 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. Defects in AIRE are a cause of autoimmune poly- endocrinopathy candidiasis ectodermal dystrophy (APS1). An autosomal recessive disease characterized by the combination of chronic mucocutaneous candidiasis, hypoparathyroidism and Addison disease. Symptoms of mucocutaneous candidiasis manifest first, followed by hypotension or fatigue occurring as a result of Addison disease. APS1 is associated with other autoimmune disorders including diabetes mellitus, vitiligo, alopecia, hepatitis, pernicious anemia and primary hypothyroidism. 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. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Transcription factor

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: cytoplasm; nucleus

Molecular Function: identical protein binding; protein binding; histone binding; zinc ion binding; translation regulator activity; chromatin binding

Biological Process: regulation of translation; regulation of transcription, DNA-dependent; transcription, DNA-dependent; positive regulation of transcription, DNA-dependent; positive regulation of transcription from RNA polymerase II promoter; immune response; humoral immune response

Disease: Autoimmune Polyendocrine Syndrome, Type I, With Or Without Reversible Metaphyseal Dysplasia