FITC标记的细胞外基质蛋白FREM2抗体
产品名称: FITC标记的细胞外基质蛋白FREM2抗体
英文名称: Anti-FREM2/FITC
产品编号: HZ-10929R-FITC
产品价格: null
产品产地: 中国/上海
品牌商标: HZbscience
更新时间: 2023-08-17T10:24:20
使用范围: ICC=1:50-200 IF=1:50-200
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Rabbit Anti-FREM2/FITC Conjugated antibody
FITC标记的细胞外基质蛋白FREM2抗体
英文名称 | Anti-FREM2/FITC |
中文名称 | FITC标记的细胞外基质蛋白FREM2抗体 |
别 名 | DKFZp781I048; ECM3 homolog; FRAS1-related extracellular matrix protein 2; FREM 2; Frem2; FREM2_HUMAN; KIAA1074. |
规格价格 | 100ul/2980元 购买 大包装/询价 |
说 明 书 | 100ul |
研究领域 | 心血管 细胞生物 发育生物学 信号转导 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | |
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 | 346kDa |
性 状 | Lyophilized or Liquid |
浓 度 | 2mg/1ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human FREM2 |
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: 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 |
FREM2是一种3169氨基酸单程I型膜蛋白,定位于细胞膜的细胞外侧,包含5个Calx-β结构域和12个CSPG重复序列。FREM2作为细胞外基质蛋白发挥作用,是维持皮肤和肾上皮细胞所必需的,并且被认为参与表皮粘附事件。编码FREM2的基因的缺陷或突变与人类染色体13有关,Fraser综合征是一种多系统畸形,其特征是耳畸形、先天性心脏缺陷和皮肤并指。FRFM2存在多个交替剪接的异构体。