Goat AIRE Polyclonal Antibody | anti-AIRE antibody

Goat Polyclonal to Human AIRE

Cat. Num. AAA241634

• Gene Names: AIRE; APS1; APSI; PGA1; AIRE1; APECED
• Reactivity: Gorilla, Human
• Applications: Immunohistochemistry, ELISA, immunoassay, Radioimmunoassay
• Purity: Immunoaffinity Purified
• Synonyms: AIRE; Polyclonal Antibody; Goat Polyclonal to Human AIRE; Anti-AIRE Antibody (C-Terminus) IHC-plus; APSI; AIRE1; Autoimmune regulator; APECED; APS1; APECED protein; PGA1; Human AIRE; anti-AIRE antibody

• Host: Goat
• Reactivity: Gorilla, Human
• Clonality: Polyclonal
• Specificity: Human AIRE. This antibody is expected to recognize isoforms 1 and 2 (NP_000374.1 and NP_000649.1 respecively).
• Purity/Purification: Immunoaffinity Purified
• Form/Format: Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide
• Concentration: 0.5 mg/ml (varies by lot)
• Sequence Length: 545

• Applicable Applications for anti-AIRE antibody: Immunohistochemistry (IHC - Paraffin), ELISA (EIA), Radioimmunoassay (RIA)
• Application Notes: ELISA (1:32000), IHC-P (2.5 ug/ml), RIA (0.01 - 0.03 ug/ml); Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti...
• Target Species: Human
• Immunogen Description: Synthetic peptide C-QSMARPAAPFPS from the C-terminus of human AIRE (NP_000374.1, NP_000649.1). Percent identity by BLAST analysis: Human, Gorilla (100%).
• Immunogen Type: Synthetic peptide
• Immunogen: AIRE antibody was raised against synthetic peptide C-QSMARPAAPFPS from the C-terminus of human AIRE (NP_000374.1, NP_000649.1). Percent identity by BLAST analysis: Human, Gorilla (100%).
• Antigen Modification: C-Terminus
• Preparation and Storage: Store at -20 degree C. Minimize freezing and thawing.

Immunohistochemistry (IHC)

(Anti-AIRE antibody IHC of human thymus. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.)

Related Product Information for anti-AIRE antibody

The AIRE gene function is not well defined, however the protein does contain zinc finger motifs suggestive of a transcription factor. The protein (isoform 1) is localized to both the nucleus and cytoplasm. Three splice variant mRNAs products have been described [1]. The longer AIRE-1 mRNA appears to be more abundant and includes exons 1 through 14. Splice variant AIRE-2 includes a portion of the non-coding region of exon 1, an alternatively spliced longer exon 8, plus exons 9 through 14.

NCBI and Uniprot Product Information

• NCBI GI #: 4557291
• NCBI GeneID: 326
• NCBI Accession #: NP_000374.1
• NCBI GenBank Nucleotide #: NM_000383.3
• UniProt Accession #: O43918; O43922; O43932; O75745; B2RP50
• Molecular Weight: 36,501 Da
• NCBI Official Full Name: autoimmune regulator
• 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; transcription, DNA-dependent; regulation of transcription, DNA-dependent; positive regulation of transcription, DNA-dependent; immune response; positive regulation of transcription from RNA polymerase II promoter; humoral immune response

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