merck millipore,默克密理博,09-456,Anti-AIRE Antibody

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merck millipore,默克密理博,09-456,Anti-AIRE Antibody
产品名称：Anti-AIRE Antibody
产品型号：09-456
Detect AIRE using this Anti-AIRE Antibody validated for use in WB.

merck millipore,默克密理博,09-456,Anti-AIRE Antibody

产品介绍

merck millipore,默克密理博,09-456,Anti-AIRE Antibody

重要规格表

品种反应性	主要应用	宿主	格式	抗体类型
M	WB	Rb	Affinity Purified	Polyclonal Antibody

描述
产品目录编号	09-456
品牌系列	Upstate
商名	Upstate
描述	Anti-AIRE Antibody
Alternate Names	autoimmune regulator (autoimmune polyendocrinopathy candidiasis<br />ectodermal dystrophy) autoimmune regulator (APECED protein) autoimmune polyendocrinopathy candidiasis ectodermal dystrophy 2 <br />autoimmune regulator <br /> Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy<br />protein APECED protein
背景信息	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).

产品信息
格式	Affinity Purified
控制	Murine thymus tissue lysate
演示	100 μg of immunoaffinity purified rabbit polyclonal IgG in 173 μl of 0.02M PBS, 0.25M NaCl containing 0.1% sodium azide

应用
应用	Detect AIRE using this Anti-AIRE Antibody validated for use in WB.
主要应用	Western Blotting
应用说明	Suggested dilutions: Western blot: 1:1000

生物信息
免疫原品种	KLH-conjugated synthetic peptide corresponding to amino acids 162-181 of mouse AIRE
表位	a.a. 162-181
宿主	Rabbit
特异性	Recognizes murine AIRE, Mr 58 kDa (calculated). Appears at ~65 kDa in Western blots.
同种型	IgG
品种反应性	Mouse
Species Reactivity Note	Mouse. Likely to cross-react with rat, based on sequence homology. Cross-reactivity with other species has not been tested
抗体类型	Polyclonal Antibody
Entrez基因编号	NM_000658.1 NM_000383.2
Entrez基因汇总	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).
基因符号	APECED AIRE AIRE1 PGA1 APSI APS1 PGAI
纯化方法	Immunoaffinity purified
UniProt编号	O43918
UniProt汇总	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.

产品使用声明
质量保证	Routinely evaluated by Western Blot analysis. Western Blot: A 1:1000 dilution of this lot detected AIRE in murine thymus lysate
使用声明	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.

储存和货运信息
存储条件	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.

储存和货运信息

存储条件

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.

包装信息
数量	100 µg

merck millipore,默克密理博,09-456,Anti-AIRE Antibody

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