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

AIRE Rabbit pAb

Cat. Num. AAA8544571

• Gene Names: AIRE; APS1; APSI; PGA1; AIRE1; APECED
• Reactivity: Human, Mouse
• Applications: Western Blot, Immunofluorescence
• Purity: Affinity Purified
• Synonyms: AIRE; Polyclonal Antibody; AIRE Rabbit pAb; APS1; APSI; PGA1; AIRE1; APECED; anti-AIRE antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity Purified
• Form/Format: PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3.
• Sequence Length: 545

• Applicable Applications for anti-AIRE antibody: Western Blot (WB), Immunofluorescence (IF)
• Application Notes: WB: 1:500-1:2000; IF: 1:50-1:200
• Immunogen: A synthetic peptide of human AIRE
• Cellular Localization: Cytoplasm, Nucleus
• Preparation and Storage: Store at -20 degree C. Avoid freeze/thaw cycles.

Related Product Information for anti-AIRE antibody

Transcription factor playing an essential role to promote self-tolerance in the thymus by regulating the expression of a wide array of self-antigens that have the commonality of being tissue-restricted in their expression pattern in the periphery, called tissue restricted antigens (TRA) (PubMed:26084028). 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. Mainly expressed by medullary thymic epithelial cells (mTECs), induces the expression of thousands of tissue-restricted proteins, which are presented on major histocompatibility complex class I (MHC-I) and MHC-II molecules to developing T-cells percolating through the thymic medulla (PubMed:26084028). Also induces self-tolerance through other mechanisms such as the regulation of the mTEC differentiation program. Controls the medullary accumulation of thymic dendritic cells and the development of regulatory T-cell through the regulation of XCL1 expression. Regulates the production of CCR4 and CCR7 ligands in medullary thymic epithelial cells and alters the coordinated maturation and migration of thymocytes. In thimic B-cells, allows the presentation of licensing-dependent endogenous self-anitgen for negative selection. In secondary lymphoid organs, induces functional inactivation of CD4+ T-cells. Expressed by a distinct bone marrow-derived population, induces self-tolerance through a mechanism that does not require regulatory T-cells and is resitant to innate inflammatory stimuli.

NCBI and Uniprot Product Information

• NCBI GI #: 4557291
• NCBI GeneID: 326
• NCBI Accession #: NP_000374.1
• NCBI GenBank Nucleotide #: NP_000374.1
• UniProt Accession #: O43918
• Molecular Weight: 55kDa
• 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
• 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