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货号： bs-13213R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-13213R-PE-Cy7
- 英文名称
- Anti-FREM1/PE-Cy7
- 中文名称
- PE-Cy7标记的细胞外基质蛋白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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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
- Human, Mouse, Rat, Dog, Horse,
- 产品应用
- 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
- 242kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human FREM1
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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.
- 产品介绍
- background:
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.
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
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.