| 产品编号 | bs-12166R-FITC |
| 英文名称 | Rabbit Anti-Fibrillin 2/FITC Conjugated antibody |
| 中文名称 | FITC标记的原纤维蛋白2抗体 |
| 别 名 | CCA; congenital contractural arachnodactyly (Marfanoid-like); DA9; FBN2; FBN2_HUMAN; fibrillin 2 (congenital contractural arachnodactyly); Fibrillin-2. |
| 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 神经生物学 信号转导 结合蛋白 细胞外基质 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
| 产品应用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 311kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Fibrillin 2 |
| 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: Extracellular glycoproteins fibrillin-1 and -2 are major components of connective tissue microfibrils. Fibrillin-2 containing microfibrils regulate the early process of elastic fiber assembly in tissue. Mutations in the fibrillin-2 gene resulting in impaired assembly of fibrillin-2 may lead to molecular congenital contractural arachnodactyly. Fibrillin-2 constitutes the backbone of microfibrils which insert directly into the lamina densa of basement membranes. Epithelial cells primarily deposit fibrillin into the extracellular matrix in a nonfibrillar form. Mutations in the 8-cysteine motif of Fibrillin-2 alters its binding to microfibril-associated glycoprotein-1 (MAGP-1), which may increase the severity of congenital contractural arachnodactyly. Function: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Subcellular Location: Secreted DISEASE: Defects in FBN2 are the cause of congenital contractural arachnodactyly (CCA) [MIM:121050]; also known as Beals syndrome or distal arthrogryposis type 9 (DA9). CCA is a rare, autosomal dominant connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Phenotypically similar to Marfan syndrome, CCA does not affect the aorta and the eyes. Similarity: Belongs to the fibrillin family. Contains 47 EGF-like domains. Contains 9 TB (TGF-beta binding) domains. Database links: Entrez Gene: 2201 Human Entrez Gene: 100047082 Mouse Entrez Gene: 14119 Mouse Omim: 121050 Human SwissProt: P35556 Human SwissProt: Q61555 Mouse Unigene: 519294 Human Unigene: 20271 Mouse Unigene: 22906 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
