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Rabbit Anti-C2orf71/BF594 Conjugated antibody (bs-15158R-BF594)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-15158R-BF594
英文名稱(chēng)1 Rabbit Anti-C2orf71/BF594 Conjugated antibody
中文名稱(chēng) BF594標(biāo)記的2號(hào)染色體開(kāi)放閱讀框71抗體
別    名 Chromosome 2 open reading frame 71; Uncharacterized protein C2orf71; CB071_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng)
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 140kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human C2orf71
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:
The protein encoded by this gene is highly expressed in photoreceptors and may associate with the primary cilium of the outer segment. The encoded protein appears to undergo post-translational lipid modification. Nonsense and missense variants of this gene appear to cause a recessive form of retinitis pigmentosa. [provided by RefSeq, Jun 2010]

Function:
May play an important role in the development of normalvision.

Subcellular Location:
Cell projection, cilium, photoreceptor outersegment.

Tissue Specificity:
Specifically expressed in retina.

DISEASE:
Defects in C2orf71 are the cause of retinitis pigmentosatype 54 (RP54) [MIM:613428]. A retinal dystrophy belonging to thegroup of pigmentary retinopathies. RP is characterized by retinalpigment deposits visible on fundus examination and primary loss ofrod photoreceptor cells followed by secondary loss of conephotoreceptors. Patients typically have night vision blindness andloss of midperipheral visual field. As their condition progresses,they lose their far peripheral visual field and eventually centralvision as well.

Database links:

Entrez Gene: 388939 Human

Omim: 613425 Human

SwissProt: A6NGG8 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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