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Rabbit Anti-FREM2  antibody (bs-10929R)  
~~~促銷(xiāo)代碼KT202411~~~
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說(shuō)明書(shū): 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-10929R
英文名稱 Rabbit Anti-FREM2  antibody
中文名稱 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.  
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) (predicted: Human,Mouse,Rat,Rabbit,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 346kDa
細(xì)胞定位 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FREM2: 1231-1330/3169 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 Preservative: 0.02% Proclin300, Constituents: 1% BSA, 0.01M PBS, pH7.4.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q5SZK8

Gene ID:
341640

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



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