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Rabbit Anti-IFT80/BF647 Conjugated antibody (bs-15564R-BF647)
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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-15564R-BF647
英文名稱(chēng) Rabbit Anti-IFT80/BF647 Conjugated antibody
中文名稱(chēng) BF647標(biāo)記的細(xì)胞纖毛內(nèi)轉(zhuǎn)運(yùn)同源蛋白80抗體
別    名 ATD2; Ift80; IFT80_HUMAN; Intraflagellar transport 80 homolog (Chlamydomonas); Intraflagellar transport protein 80 homolog; KIAA1374; WD repeat domain 56; WD repeat-containing protein 56; WDR56.   
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        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)  轉(zhuǎn)運(yùn)蛋白  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, )
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 88kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IFT80
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:
IFT80 is a encoded by this gene is part of the intraflagellar transport complex B and is necessary for the function of motile and sensory cilia. Defects in this gene are a cause of asphyxiating thoracic dystrophy 2 (ATD2). Three transcript variants encoding two different isoforms have been found for this gene.

Function:
Component of the intraflagellar transport (IFT) complex B, which is essential for the development and maintenance of motile and sensory cilia.

Subcellular Location:
Cytoplasm. Cytoplasm, cytoskeleton, cilium basal body (By similarity). Cytoplasm, cytoskeleton, cilium axoneme (By similarity). Note=Basal body and ciliary axoneme (By similarity).

DISEASE:
Asphyxiating thoracic dystrophy 2 (ATD2) [MIM:611263]: An autosomal recessive chondrodysplasia characterized by a severely constricted thoracic cage, short-limbed short stature, and polydactyly. It often leads to death in infancy because of respiratory insufficiency. Retinal degeneration, cystic renal disease and hepatic disease can be present in affected individuals who survive early childhood. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Contains 7 WD repeats.

Database links:
UniProtKB/Swiss-Prot: Q9P2H3.3

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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