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Rat Anti-AIRE Recombinant Antibody (V2-12554) (CBMAB-1044-CN)

This product is a rat antibody that recognizes AIRE of mouse. The antibody 609930 can be used for immunoassay techniques such as: WB, CyTOF, FC.
See all AIRE antibodies

Summary

Host Animal
Rat
Specificity
Mouse
Clone
V2-12554
Antibody Isotype
IgG2a
Application
WB, IHC-F

Basic Information

Immunogen
E. coli-derived recombinant mouse AIRE Ser476-Ser552.
Host Species
Rat
Specificity
Mouse
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1 μg/ml
FC2.5 µg/10^6 cells

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Buffer
PBS, Trehalose.
Preservative
None
Concentration
Batch dependent
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Autoimmune Regulator
Introduction
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) (Probable). The protein 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. It also binds to nucleosomes (By similarity). 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'This protein functions as a sensor of histone H3 modifications that are important for the epigenetic regulation of gene expression. This gene is 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 (By similarity).
Entrez Gene ID
11634
UniProt ID
Q9Z0E3
Alternative Names
autoimmune regulator; APECED protein homolog; autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein homolog; autoimmune regulator AIRE1a
Function
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 (By similarity). 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 (By similarity).
Biological Process
Central tolerance induction to self antigen Source: UniProtKB
Humoral immune response Source: GO_Central
Immune response Source: ProtInc
Negative thymic T cell selection Source: GO_Central
Peripheral T cell tolerance induction Source: UniProtKB
Positive regulation of chemokine production Source: UniProtKB
Positive regulation of transcription, DNA-templated Source: UniProtKB
Positive regulation of transcription by RNA polymerase II Source: NTNU_SB
Regulation of thymocyte migration Source: UniProtKB
Regulation of transcription, DNA-templated Source: UniProtKB
Thymus epithelium morphogenesis Source: UniProtKB
Cellular Location
Cytoplasm; Nucleus. 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).
Involvement in disease
Autoimmune polyendocrine syndrome 1, with or without reversible metaphyseal dysplasia (APS1): The disease is caused by variants affecting the gene represented in this entry. 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 (PubMed:14974083). Heterozygous mutations within the PHD1 domain have dominant-negatif effects and cause organ-specific autoimmune diseases (PubMed:26084028). Patients harbor extremely high-affinity, neutralizing autoantibodies, particularly against specific cytokines such as type I interferons which could protect them from some types of autoimmune diseases, like type I diabetes (PubMed:27426947).
A rare 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.
PTM
Phosphorylated. Phosphorylation could trigger oligomerization.

Warren, B. D., Ahn, S. H., Brittain, K., Nanjappa, M. K., Wang, H., Wang, J., ... & Petroff, M. G. (2021). Multiple lesions contribute to infertility in males lacking autoimmune regulator (AIRE). The American Journal of Pathology.

Fawzy, M. S., & Toraih, E. A. (2020). Analysis of the autoimmune regulator (AIRE) gene variant rs2075876 (G/A) association with breast cancer susceptibility. Journal of clinical laboratory analysis, 34(9), e23365.

Melo‐Lima, B. L., Poras, I., Passos, G. A., Carosella, E. D., Donadi, E. A., & Moreau, P. (2019). The Autoimmune Regulator (Aire) transactivates HLA‐G gene expression in thymic epithelial cells. Immunology, 158(2), 121-135.

Eriksson, D., Bianchi, M., Landegren, N., Dalin, F., Skov, J., Hultin-Rosenberg, L., ... & Kämpe, O. (2018). Common genetic variation in the autoimmune regulator (AIRE) locus is associated with autoimmune Addison’s disease in Sweden. Scientific reports, 8(1), 1-11.

Zhao, B., Chang, L., Fu, H., Sun, G., & Yang, W. (2018). The role of autoimmune regulator (AIRE) in peripheral tolerance. Journal of immunology research, 2018.

Conteduca, G., Indiveri, F., Filaci, G., & Negrini, S. (2018). Beyond APECED: an update on the role of the autoimmune regulator gene (AIRE) in physiology and disease. Autoimmunity reviews, 17(4), 325-330.

Abbott, J. K., Huoh, Y. S., Reynolds, P. R., Yu, L., Rewers, M., Reddy, M., ... & Gelfand, E. W. (2018). Dominant-negative loss of function arises from a second, more frequent variant within the SAND domain of autoimmune regulator (AIRE). Journal of autoimmunity, 88, 114-120.

Nishijima, H., Kajimoto, T., Matsuoka, Y., Mouri, Y., Morimoto, J., Matsumoto, M., ... & Matsumoto, M. (2018). Paradoxical development of polymyositis-like autoimmunity through augmented expression of autoimmune regulator (AIRE). Journal of autoimmunity, 86, 75-92.

Herzig, Y., Nevo, S., Bornstein, C., Brezis, M. R., Ben-Hur, S., Shkedy, A., ... & Abramson, J. (2017). Transcriptional programs that control expression of the autoimmune regulator gene Aire. Nature immunology, 18(2), 161-172.

Guha, M., Saare, M., Maslovskaja, J., Kisand, K., Liiv, I., Haljasorg, U., ... & Peterson, P. (2017). DNA breaks and chromatin structural changes enhance the transcription of autoimmune regulator target genes. Journal of Biological Chemistry, 292(16), 6542-6554.

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For research use only. Not intended for any clinical use.

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