Anti-AIRE (rabbit polyclonal)

Species Reactivity	Key Applications	Host	Format	Antibody Type
M	WB	Rb	Affinity Purified	Polyclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

Affinity Purified

Polyclonal Antibody

Description
Catalogue Number	09-456
Brand Family	Upstate
Trade Name	Upstate
Description	Anti-AIRE Antibody
Alternate Names	APECED protein Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy protein autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2 autoimmune regulator autoimmune regulator (APECED protein) autoimmune regulator (autoimmune polyendocrinopathy candidiasis ectodermal dystrophy)
Background Information	AIRE (Autoimmune Regulator) is a human gene that is expressed primarily in the thymus. It causes transcription of a wide selection of organ-specific genes, reducing the threat of autoimmunity occurring by allowing the elimination of autoreactive T cells via negative selection. Defects in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendo-crinopathy-candidiasis-ectodermal dystrophy (APECED). Disruption of AIRE results in the development of a range of autoimmune diseases, the most common clinical conditions in the syndrome are hypoparathyroidism, primary adrenocortical failure and chronic mucocutaneous candidiasis. Aire functions through initiating the transcription of a diverse set of self-antigens, such as insulin, in the thymus. This expression then allows maturing thymocytes to become tolerant towards peripheral organs, thereby suppressing autoimmune disease. Aire is atranscriptional regulator protein that binds to DNA as dimer and tetramer, but not as a monomer. It forms nuclear bodies and interacts with the transcriptional coactivator CBP (CREBBP). 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).

Description

Catalogue Number

09-456

Brand Family

Upstate

Trade Name

Upstate

Description

Anti-AIRE Antibody

Alternate Names

APECED protein
Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy
protein
autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2
autoimmune regulator
autoimmune regulator (APECED protein)
autoimmune regulator (autoimmune polyendocrinopathy candidiasis
ectodermal dystrophy)

Background Information

AIRE (Autoimmune Regulator) is a human gene that is expressed primarily in the thymus. It causes transcription of a wide selection of organ-specific genes, reducing the threat of autoimmunity occurring by allowing the elimination of autoreactive T cells via negative selection. Defects in this gene cause the rare autosomal-recessive systemic autoimmune disease termed autoimmune polyendo-crinopathy-candidiasis-ectodermal dystrophy (APECED). Disruption of AIRE results in the development of a range of autoimmune diseases, the most common clinical conditions in the syndrome are hypoparathyroidism, primary adrenocortical failure and chronic mucocutaneous candidiasis. Aire functions through initiating the transcription of a diverse set of self-antigens, such as insulin, in the thymus. This expression then allows maturing thymocytes to become tolerant towards peripheral organs, thereby suppressing autoimmune disease. Aire is atranscriptional regulator protein that binds to DNA as dimer and tetramer, but not as a monomer. It forms nuclear bodies and interacts with the transcriptional coactivator CBP (CREBBP). 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).

Product Information
Format	Affinity Purified
Control	Murine thymus tissue lysate
Presentation	100 μg of immunoaffinity purified rabbit polyclonal IgG in 173 μl of 0.02M PBS, 0.25M NaCl containing 0.1% sodium azide

Product Information

Format

Affinity Purified

Control

Murine thymus tissue lysate

Presentation

100 μg of immunoaffinity purified rabbit polyclonal IgG in 173 μl of 0.02M PBS, 0.25M NaCl containing 0.1% sodium azide

Applications
Application	Detect AIRE using this Anti-AIRE Antibody validated for use in WB.
Key Applications	Western Blotting
Application Notes	Suggested dilutions: Western blot: 1:1000

Applications

Application

Detect AIRE using this Anti-AIRE Antibody validated for use in WB.

Key Applications

Western Blotting

Application Notes

Suggested dilutions:
Western blot: 1:1000

Biological Information
Immunogen	KLH-conjugated synthetic peptide corresponding to amino acids 162-181 of mouse AIRE
Epitope	a.a. 162-181
Host	Rabbit
Specificity	Recognizes murine AIRE, Mr 58 kDa (calculated). Appears at ~65 kDa in Western blots.
Isotype	IgG
Species Reactivity	Mouse
Species Reactivity Note	Mouse. Likely to cross-react with rat, based on sequence homology. Cross-reactivity with other species has not been tested
Antibody Type	Polyclonal Antibody
Entrez Gene Number	NM_000383.2 NM_000658.1
Entrez Gene Summary	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).
Gene Symbol	AIRE AIRE1 PGA1 APSI APS1 PGAI APECED
Purification Method	ImmunoAffinity Purified
UniProt Number	O43918
UniProt Summary	FUNCTION: SwissProt: O43918 # Probable transcriptional regulator protein that binds to DNA as dimer and 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. May be involved in immune regulation. SIZE: 545 amino acids; 57727 Da SUBUNIT: Homodimer and homotetramer. Interacts with CREBBP. SUBCELLULAR LOCATION: Nucleus. Cytoplasm. Note=Associated with tubular structures and in discrete nuclear dots resembling ND10 nuclear bodies. May shuttle between nucleus and cytoplasm. TISSUE SPECIFICITY: Widely expressed. Expressed at higher level in thymus (medullary epithelial cells and monocyte-dendritic cells), pancreas, adrenal cortex, and testis. Expressed at lower level in the spleen, fetal liver and lymph nodes. Isoforms 2 and 3 seem to be less frequently expressed than isoform 1, if at all. DOMAIN: SwissProt: O43918 The L-X-X-L-L repeats may be implicated in binding to nuclear receptors. & The HSR domain is required for localization on tubular structures (N-terminal part) and for homodimerization. & Disruption of the first PHD domain has been shown to lead to reduced transcriptional activity and to localization of the protein mainly in the cytoplasm in small granules. While the PHD zinc fingers are necessary for the transactivation capacity of the protein, other regions also modulate this function. PTM: Phosphorylated. Phosphorylation could trigger oligomerization. DISEASE: SwissProt: O43918 # Defects in AIRE are a cause of autoimmune poly- endocrinopathy candidiasis ectodermal dystrophy (APECED) [MIM:240300]; also known as autoimmune polyglandular syndrome type I (APS-1). APECED is an autosomal recessive disease characterized by: (1) autoimmune polyendocrinopathies: hypoparathyroidism, adrenocortical failure, IDDM, gonadal failure, hypothyroidism, pernicious anemia, and hepatitis; (2) chronic mucocutaneous candidiasis; (3) ectodermal dystrophies: vitiligo, alopecia, keratopathy, dystrophy of dental enamel, nails and tympanic membranes. In addition, a high proportion of patients develop squamous cell carcinoma of the oral mucosa. The disease is reported worldwide but is exceptionally prevalent among the Finnish population (incidence 1:25000) and the Iranian jews (incidence 1:9000). & 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. SIMILARITY: Contains 1 HSR domain. & Contains 2 PHD-type zinc fingers. & Contains 1 SAND domain.
Molecular Weight	65 kDa

Biological Information

Immunogen

KLH-conjugated synthetic peptide corresponding to amino acids 162-181 of mouse AIRE

Epitope

a.a. 162-181

Host

Rabbit

Specificity

Recognizes murine AIRE, Mr 58 kDa (calculated). Appears at ~65 kDa in Western blots.

Isotype

IgG

Species Reactivity

Mouse

Species Reactivity Note

Mouse. Likely to cross-react with rat, based on sequence homology. Cross-reactivity with other species has not been tested

Antibody Type

Polyclonal Antibody

Entrez Gene Number

Entrez Gene Summary

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).

Gene Symbol

AIRE
AIRE1
PGA1
APSI
APS1
PGAI
APECED

Purification Method

ImmunoAffinity Purified

UniProt Number

O43918

UniProt Summary

FUNCTION: SwissProt: O43918 # Probable transcriptional regulator protein that binds to DNA as dimer and 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. May be involved in immune regulation.
SIZE: 545 amino acids; 57727 Da
SUBUNIT: Homodimer and homotetramer. Interacts with CREBBP.
SUBCELLULAR LOCATION: Nucleus. Cytoplasm. Note=Associated with tubular structures and in discrete nuclear dots resembling ND10 nuclear bodies. May shuttle between nucleus and cytoplasm.
TISSUE SPECIFICITY: Widely expressed. Expressed at higher level in thymus (medullary epithelial cells and monocyte-dendritic cells), pancreas, adrenal cortex, and testis. Expressed at lower level in the spleen, fetal liver and lymph nodes. Isoforms 2 and 3 seem to be less frequently expressed than isoform 1, if at all.
DOMAIN: SwissProt: O43918 The L-X-X-L-L repeats may be implicated in binding to nuclear receptors. & The HSR domain is required for localization on tubular structures (N-terminal part) and for homodimerization. & Disruption of the first PHD domain has been shown to lead to reduced transcriptional activity and to localization of the protein mainly in the cytoplasm in small granules. While the PHD zinc fingers are necessary for the transactivation capacity of the protein, other regions also modulate this function.
PTM: Phosphorylated. Phosphorylation could trigger oligomerization.
DISEASE: SwissProt: O43918 # Defects in AIRE are a cause of autoimmune poly- endocrinopathy candidiasis ectodermal dystrophy (APECED) [MIM:240300]; also known as autoimmune polyglandular syndrome type I (APS-1). APECED is an autosomal recessive disease characterized by: (1) autoimmune polyendocrinopathies: hypoparathyroidism, adrenocortical failure, IDDM, gonadal failure, hypothyroidism, pernicious anemia, and hepatitis; (2) chronic mucocutaneous candidiasis; (3) ectodermal dystrophies: vitiligo, alopecia, keratopathy, dystrophy of dental enamel, nails and tympanic membranes. In addition, a high proportion of patients develop squamous cell carcinoma of the oral mucosa. The disease is reported worldwide but is exceptionally prevalent among the Finnish population (incidence 1:25000) and the Iranian jews (incidence 1:9000). & 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.
SIMILARITY: Contains 1 HSR domain. & Contains 2 PHD-type zinc fingers. & Contains 1 SAND domain.

Molecular Weight

65 kDa

Product Usage Statements
Quality Assurance	Routinely evaluated by Western Blot analysis. Western Blot: A 1:1000 dilution of this lot detected AIRE in murine thymus lysate
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Quality Assurance

Routinely evaluated by Western Blot analysis.

Western Blot:
A 1:1000 dilution of this lot detected AIRE in murine thymus lysate

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	2 years at -20°C from date of shipment. Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance. Note: Variability in freezer temperatures below -20°C may cause glycerol-containing solutions to become frozen during storage.

Storage and Shipping Information

Storage Conditions

2 years at -20°C from date of shipment. Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance. Note: Variability in freezer temperatures below -20°C may cause glycerol-containing solutions to become frozen during storage.

Packaging Information
Material Size	100 µg

Packaging Information

Material Size

100 µg

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Anti-AIRE (rabbit polyclonal)

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