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Rabbit Anti-FRAS1/BF594 Conjugated antibody (bs-13212R-BF594)
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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-13212R-BF594
英文名稱 Rabbit Anti-FRAS1/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的細(xì)胞外基質(zhì)蛋白FRAS1抗體
別    名 Extracellular matrix protein FRAS1; Fras 1; Fras1; FRAS1_HUMAN; Fraser syndrome 1.  
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 心血管  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞外基質(zhì)  
抗體來(lái)源 Rabbit
克隆類型 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 440kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FRAS1
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:
This gene encodes an extracellular matrix protein that appears to function in the regulation of epidermal-basement membrane adhesion and organogenesis during development. Mutations in this gene cause Fraser syndrome, a multisystem malformation that can include craniofacial, urogenital and respiratory system abnormalities. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2009].

Subcellular Location:
Cell membrane; Single-pass type I membrane protein; Extracellular side (Potential).

Tissue Specificity:
Expressed in many adult tissues, with highest levels in kidney, pancreas and thalamus. Relatively high expression was also detected in fetal kidney and heart.

DISEASE:
Defects in FRAS1 are a cause of Fraser syndrome (FRASS) [MIM:219000]. Fraser syndrome is a multisystem malformation usually comprising cryptophthalmos, cutaneous syndactyly, ear abnormalities, renal agenesis and congenital heart defects.

Similarity:
Belongs to the FRAS1 family.
Contains 5 Calx-beta domains.
Contains 12 CSPG (NG2) repeats.
Contains 14 FU (furin-like) repeats.
Contains 6 VWFC domains.

Database links:

Entrez Gene: 80144 Human

Omim: 607830 Human

SwissProt: Q86XX4 Human

Unigene: 369448 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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