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Rabbit Anti-FREM2/BF647 Conjugated antibody (bs-10929R-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-10929R-BF647
英文名稱1 Rabbit Anti-FREM2/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的細(xì)胞外基質(zhì)蛋白FREM2抗體
別    名 DKFZp781I048; ECM3 homolog; FRAS1-related extracellular matrix protein 2; FREM 2; Frem2; FREM2_HUMAN; KIAA1074.  
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  發(fā)育生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
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 346kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FREM2
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:
FREM2 is a 3,169 amino acid single-pass type I membrane protein that localizes to the extracellular side of the cell membrane and contains five Calx-beta domains, as well as 12 CSPG repeats. Functioning as an extracellular matrix protein, FREM2 is required for the maintenance of skin and renal epithelia and is also thought to be involved in epidermal adhesion events. Defects or mutations in the gene encoding FREM2, which maps to human chromsome 13, are associated with Fraser syndrome, a multisystem malformation that is characterized by ear abnormalities, congenital heart defects and cutaneous syndactyly. FREM2 exists as multiple alternatively spliced isoforms.

Function:
Extracellular matrix protein required for maintenance of the integrity of the skin epithelium and for maintenance of renal epithelia. May be required for epidermal adhesion.

Subcellular Location:
Cell membrane.

DISEASE:
Defects in FREM2 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.

Database links:

Entrez Gene: 341640 Human

Entrez Gene: 242022 Mouse

Entrez Gene: 310418 Rat

Omim: 608945 Human

SwissProt: Q5SZK8 Human

SwissProt: Q6NVD0 Mouse

Unigene: 253994 Human

Unigene: 38378 Mouse

Unigene: 63994 Rat



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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