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FREM1 Rabbit pAb (bs-13213R)  
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產品編號 bs-13213R
英文名稱 FREM1 Rabbit pAb
中文名稱 細胞外基質蛋白FREM1抗體
別    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.  
研究領域 細胞生物  發育生物學  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat,Dog,Horse)
產品應用 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 242 kDa
檢測分子量
細胞定位 細胞膜 細胞外基質 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FREM1: 1201-1300/2179 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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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