FITC标记的细胞外基质蛋白FRAS1抗体
产品名称: FITC标记的细胞外基质蛋白FRAS1抗体
英文名称: Anti-FRAS1/FITC
产品编号: HZ-13212R-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-FRAS1/FITC Conjugated antibody
FITC标记的细胞外基质蛋白FRAS1抗体
英文名称 | Anti-FRAS1/FITC |
中文名称 | FITC标记的细胞外基质蛋白FRAS1抗体 |
别 名 | Extracellular matrix protein FRAS1; Fras 1; Fras1; FRAS1_HUMAN; Fraser syndrome 1. |
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 信号转导 细胞外基质 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Human, Mouse, Rat, |
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 | 440kDa |
细胞定位 | 细胞膜 |
性 状 | Lyophilized or Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human FRAS1 |
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: This gene encodes an extracellular matrix protein that appears to function in the regulation of epidermal-basement membrane adhesion and organogenesis during development. Mutations in this gene cause Fraser syndrome, a multisystem malformation that can include craniofacial, urogenital and respiratory system abnormalities. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2009]. Subcellular Location: Cell membrane; Single-pass type I membrane protein; Extracellular side (Potential). Tissue Specificity: Expressed in many adult tissues, with highest levels in kidney, pancreas and thalamus. Relatively high expression was also detected in fetal kidney and heart. DISEASE: Defects in FRAS1 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. Contains 14 FU (furin-like) repeats. Contains 6 VWFC domains. Database links: Entrez Gene: 80144 Human Omim: 607830 Human SwissProt: Q86XX4 Human Unigene: 369448 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码一种细胞外基质蛋白,在发育过程中似乎在表皮-基底膜粘附和器官发生的调节中起作用。该基因的突变导致弗雷泽综合征,一种多系统畸形,可包括颅面、泌尿生殖和呼吸系统异常。选择性剪接导致多个转录变体。[ RefSeq,OCT 2009 ]提供。