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Rabbit Anti-FREM1  antibody (bs-13213R)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-13213R
英文名称 FREM1
中文名称 细胞外基质蛋白FREM1抗体
别    名 BC037594; BNAR; C9orf143; C9orf145; C9orf154; D430009N09; D630008K06; FLJ25461; FRAS1-related extracellular matrix protein 1; FREM 1; FREM1; FREM1_HUMAN; Heb; MOTA; Protein QBRICK; QBRICK; RGD1306981; RP11-265B7.2; RP23-410K19.1; TILRR.  
研究领域 细胞生物  发育生物学  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Dog,Horse)
产品应用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 242kDa
细胞定位 细胞膜 细胞外基质 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FREM1: 1201-1300/2179 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 FREM1 is a 2,179 amino acid protein that contains one C-type lectin domain, one Calx-beta domain and twelve CSPG repeats. Localized to the basement membrane of embryonic epidermal cells and secreted into extracellular space, FREM1 functions as an extracellular matrix protein that is essential for epidermal adhesion during embryogenesis and may also participate in epidermal differentiation. FREM1 exists as multiple alternatively spliced isoforms and is encoded by a gene which maps to human chromosome 9. Chromosome 9 contains 145 million base pairs and comprises 4% of the human genome, encoding nearly 900 genes. Hereditary hemorrhagic telangiectasia, which is characterized by harmful vascular defects, and Familial dysautonomia, are both associated with chromosome 9. Notably, chromosome 9 encompasses the largest interferon family gene cluster.

Function:
Extracellular matrix protein that plays a role in epidermal differentiation and is required for epidermal adhesion during embryonic development

Subcellular Location:
Secreted; extracellular space; extracellular matrix; basement membrane. Localizes at the basement membrane zone of embryonic epidermis and hair follicles.

DISEASE:
Defects in FREM1 are the cause of bifid nose with or without anorectal and renal anomalies (BNAR) [MIM:608980]. A bifid nose is a rare congenital deformity due to failure of the paired nasal processes to fuse to a single midline organ during early gestation. BNAR is an autosomal recessive disorder and patients usually present a bifid nose associated with renal and anorectal malformations.

Similarity:
Belongs to the FRAS1 family.
Contains 1 C-type lectin domain.
Contains 1 Calx-beta domain.
Contains 12 CSPG (NG2) repeats.

SWISS:
Q5H8C1

Gene ID:
158326

Database links:

Entrez Gene: 158326 Human

Entrez Gene: 329872 Mouse

Omim: 608944 Human

SwissProt: Q5H8C1 Human

SwissProt: Q684R7 Mouse

Unigene: 50850 Human

Unigene: 242337 Mouse



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