Goat anti-Human AIRE, Isoform 1 Polyclonal Antibody | anti-Aire antibody

AIRE, Isoform 1 (Autoimmune Regulator, Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy Protein, APECED Protein)

Cat. Num. AAA6003510

• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity Purified; Purified by immunoaffinity chromatography.
• Synonyms: AIRE; Isoform 1; Polyclonal Antibody; Isoform 1 (Autoimmune Regulator; Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy Protein; APECED Protein); Anti -AIRE; anti-Aire antibody

• Host: Goat
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human AIRE (isoform 1).
• Purity/Purification: Affinity Purified; Purified by immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.

• Applicable Applications for anti-Aire antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in ELISA, Immunohistochemistry and Western Blot.; Dilution: Immunohistochemistry (Frozen and Formalin fixed paraffin embedded): 2.5ug/ml; Western Blot: 0.5-1ug/ml; ELISA: 1:32,000
• Immunogen: Synthetic peptide corresponding to aa102-115 (C-KDVDLSQPRKGRKP) from the internal region of human AIRE (NP_000374.1).
• 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 at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Related Product Information for anti-Aire antibody

AIRE is a transcriptional regulator that is thought to be important in controlling tissue-specific expression of genes in the thymus. The precise function of the protein is not well defined. It contains zinc finger motifs. The AIRE isoform 1 (AIRE) is a splice variant and is localized to both the nucleus and cytoplasm.

Defects in this gene cause the autosomal-recessive systemic autoimmune disease termed autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).

Product Categories/Family for anti-Aire antibody

Antibodies; Abs to Transcription Factors

NCBI and Uniprot Product Information

• NCBI GI #: 6753020
• NCBI GeneID: 11634
• NCBI Accession #: NP_033776.1
• NCBI GenBank Nucleotide #: NM_009646.2
• UniProt Accession #: Q9Z0E3; Q9JLW0; Q9JLW1; Q9JLW2; Q9JLW3; Q9JLW4; Q9JLW5; Q9JLW6; Q9JLW7; Q9JLW8; Q9JLW9; Q9JLX0
• Molecular Weight: 59,042 Da
• NCBI Official Full Name: autoimmune regulator isoform 1
• NCBI Official Synonym Full Names: autoimmune regulator (autoimmune polyendocrinopathy candidiasis ectodermal dystrophy)
• NCBI Official Symbol: Aire
• NCBI Protein Information: autoimmune regulator; APECED protein homolog; autoimmune regulator AIRE1a; autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein homolog
• UniProt Protein Name: Autoimmune regulator
• Protein Family: Autoimmune regulator
• UniProt Gene Name: Aire
• UniProt Synonym Gene Names: APECED protein homolog
• UniProt Entry Name: AIRE_MOUSE

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

Cellular Component: cytoplasm; intracellular; nucleus

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

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