FITC标记的纤连蛋白4抗体
产品名称： FITC标记的纤连蛋白4抗体
英文名称： Anti-EFEMP2/FITC
产品编号： HZ-13059R-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
上海沪震实业有限公司
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 139****0749
- 传真 : 021-60345367
- 邮箱 : www.shzbio.net
Rabbit Anti-EFEMP2/FITC Conjugated antibody
FITC标记的纤连蛋白4抗体
| 英文名称 | Anti-EFEMP2/FITC |
| 中文名称 | FITC标记的纤连蛋白4抗体 |
| 别 名 | Fibulin 4; EFEMP2; EGF containing fibulin like extracellular matrix protein 2; EGF-containing fibulin-like extracellular matrix protein 2; FBLN 4; FBLN4; FBLN4_HUMAN; FIBL 4; FIBL-4; FIBL4; Fibulin4; Fibulin-4; MBP 1; MBP1; Mutant p53 binding protein 1; Protein UPH1; UPH 1; UPH1; UPH1 protein. |
| 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 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, Dog, Pig, Cow, Horse, Sheep, |
| 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 | 47kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human EFEMP2/Fibulin 4 |
| 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: Fibulin proteins contribute to normal development of elastic fiber systems in various types of organs that require elasticity, such as vasculature, lung and skin. Fibulin-4, also known as EFEMP2 (EGF-containing fibulin-like extracellular matrix protein 2), MBP1 or UPH1 is a 443 amino acid secreted protein that contains six EGF-like calcium-binding domains and belongs to the fibulin family. Expressed ubiquitously with highest expression in heart, Fibulin-4 is essential for connective tissue development and elastic fiber formation, and may also play an important role in vascular patterning and collagen biosynthesis. Defects in the gene encoding Fibulin-4 are associated with autosomal recessive cutis laxa type I (CL type I), a connective tissue disorder that is inherited in both an autosomal dominant and an autosomal recessive manner and is characterized by inelastic tissue in all affected areas of the body. Subcellular Location: Secreted. DISEASE: Defects in EFEMP2 are a cause of cutis laxa autosomal recessive type 1 (ARCL1) [MIM:219100]. Hereditary cutis laxa refers to a heterogeneous group of connective tissue disorders characterized by cutaneous abnormalities and variable systemic manifestations. The most constant clinical feature is loose skin, sagging over the face and trunk. Hereditary cutis laxa is inherited in both autosomal dominant and autosomal recessive modes. ARCL1 shows the most severe phenotype and has the poorest prognosis. In addition to the skin, internal organs enriched in elastic fibers, such as the lung and arteries, are affected Similarity: Belongs to the fibulin family. Contains 6 EGF-like domains. Database links: Entrez Gene: 30008 Human Omim: 604633 Human SwissProt: O95967 Human Unigene: 170622 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
纤维蛋白有助于需要弹性的各种器官中弹性纤维系统的正常发育，如血管、肺和皮肤。纤维蛋白-4，也称为EFEMP2（含EGF的纤维蛋白样细胞外基质蛋白2），MBP1或UPH1是一种443个氨基酸分泌的蛋白质，含有6个EGF样钙结合结构域，属于纤维蛋白家族。纤维蛋白4(Fibulin-4)广泛表达于心脏，在结缔组织发育和弹性纤维形成中起重要作用，在血管形成和胶原生物合成中也起着重要作用。纤维蛋白-4基因中的缺陷与常染色体隐性皮肤松弛I型(CL I型)相关，这是一种结缔组织疾病，以常染色体显性和常染色体隐性方式遗传，并且以身体所有受影响区域的无弹性组织为特征。