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Rabbit Anti-DUOXA2 /BF350 Conjugated antibody (bs-23964R-BF350)
訂購(gòu)熱線:400-901-9800
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-23964R-BF350
英文名稱1 Rabbit Anti-DUOXA2 /BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的雙氧化酶激活因子2抗體
別    名 Dual oxidase activator 2; Dual oxidase maturation factor 2; SIMNIPHOM; TDH5; DOXA2_HUMAN; Dual oxidase maturation factor-2; Dual oxidase activator 2..  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  通道蛋白  細(xì)胞膜受體  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, )
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 35kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DUOXA2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
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:
DUOXA2 is a 320 amino acid multi-pass membrane protein that localizes to the endoplasmic reticulum (ER) and belongs to the DUOXA family. Expressed specifically in thyroid and salivary glands, DUOXA2 is essential for the maturation and transport of DUOX2 from the ER to the plasma membrane and is also thought to play a role in the synthesis of thyroid hormone (TH). Defects in the DUOXA2 gene are associated with the pathogenesis of congenital hypothyroidism, a disorder that affects infants and is characterized by a significant decrease or a complete deficiency of TH from birth. The gene encoding DUOXA2 maps to human chromosome 15, which houses over 700 genes and comprises nearly 3% of the human genome. Angelman syndrome, Prader-Willi syndrome, Tay-Sachs disease and Marfan syndrome are all associated with defects in chromosome 15-localized genes.

Function:
Function: Required for the maturation and the transport from the endoplasmic reticulum to the plasma membrane of functional DUOX2. May play a role in thyroid hormone synthesis.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
Specifically expressed in thyroid. Also detected in salivary glands. [PTM] N-glycosylated.

DISEASE:
Defects in DUOXA2 are the cause of thyroid dyshormonogenesis 5 (TDH5) [MIM:274900]. A disorder due to thyroid dyshormonogenesis, causing hypothyroidism, goiter, and variable mental deficits derived from unrecognized and untreated hypothyroidism

Similarity:
Belongs to the DUOXA family.

Database links:

Entrez Gene: 405753 Human

Omim: 612772 Human

SwissProt: Q1HG44 Human

Unigene: 497987 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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