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Rabbit Anti-RFXANK/RBITC Conjugated antibody (bs-19843R-RBITC)
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@m.p2b3.cn
訂購(gòu)QQ:  400-901-9800
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-19843R-RBITC
英文名稱1 Rabbit Anti-RFXANK/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的調(diào)節(jié)因子X(jué)相關(guān)錨蛋白R(shí)FXANK抗體
別    名 ANKRA1; Ankyrin repeat containing regulatory factor X associated protein; Ankyrin repeat family A protein 1; BLS; DNA-binding protein RFXANK; F14150_1; MGC138628; Regulatory factor X associated ankyrin containing protein; Regulatory factor X subunit B; Regulatory factor X-associated ankyrin-containing protein; RFX-B; RFXANK; RFXB; RFXB delta 4; RFXK_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  結(jié)合蛋白  細(xì)胞類型標(biāo)志物  淋巴細(xì)胞  t-淋巴細(xì)胞  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Sheep, Chimpanzee, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 28kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human RFXANK
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
Defects in RFXANK are a cause of bare lymphocyte syndrome type 2 (BLS2) [MIM:209920]; also known as hereditary MHC class II deficiency or HLA class II-deficient combined immunodeficiency. BLS2 is a severe combined immunodeficiency disease with early onset. It is characterized by a profound defect in constitutive and interferon-gamma induced MHC II expression, absence of cellular and humoral T-cell response to antigen challenge, hypogammaglobulinemia and impaired antibody production. The consequence include extreme susceptibility to viral, bacterial and fungal infections.

Function:
Activates transcription from class II MHC promoters. Activation requires the activity of the MHC class II transactivator (MHC2TA). May regulate other genes in the cell. RFX binds the X1 box of MHC-II promoters. Isoform RFX-B-delta5 is not involved in the positive regulation of MHC class II genes.

Subunit:
The RFX heterotetrameric complex consists of 2 molecules of RFX5 and one each of RFXAP and RFX-B/RFXANK; with each subunit representing a separate complementation group. RFX forms cooperative DNA binding complexes with X2BP and CBF/NF-Y. RFX associates with MHC2TA to form an active transcriptional complex.

Subcellular Location:
Nucleus.

Tissue Specificity:
Ubiquitous.

DISEASE:
Defects in RFXANK are a cause of bare lymphocyte syndrome type 2 (BLS2) [MIM:209920]; also known as hereditary MHC class II deficiency or HLA class II-deficient combined immunodeficiency. BLS2 is a severe combined immunodeficiency disease with early onset. It is characterized by a profound defect in constitutive and interferon-gamma induced MHC II expression, absence of cellular and humoral T-cell response to antigen challenge, hypogammaglobulinemia and impaired antibody production. The consequence include extreme susceptibility to viral, bacterial and fungal infections.

Similarity:
Contains 5 ANK repeats.

Database links:
Entrez Gene: 8625 Human

Omim: 603200 Human

SwissProt: O14593 Human

Unigene: 153629 Human

Unigene: 296776 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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