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- Bioss
- bs-12166R-Bio
- 2025年09月30日
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100ul
| 产品编号 | bs-12166R-Bio |
| 英文名称 | Fibrillin 2 Rabbit pAb, Biotin conjugated |
| 中文名称 | Biotin标记的原纤维蛋白2抗体 |
| 英文别名 | CCA; congenital contractural arachnodactyly(Marfanoid-like); DA9; FBN2; FBN2_HUMAN; fibrillin 2(congenital contractural arachnodactyly); Fibrillin-2. |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human Fibrillin 2 |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Neuroscience > Neurotransmission > Calcium Signaling > Calcium Binding Proteins Signal Transduction > Cytoskeleton / ECM > Extracellular Matrix > ECM Proteins > Extended Fibrils Signal Transduction > Signaling Pathway > Calcium Signaling > Calcium Binding Proteins |
| 亚细胞定位 | Secreted |
| 相似性 | Belongs to the fibrillin family. _x000D_ Contains 47 EGF-like domains. _x000D_ Contains 9 TB (TGF-beta binding) domains. |
| 功能 | 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. |
| 保存条件 | Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
| 应用 | 推荐稀释比例 |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {IF} | {1:100-500} |
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Fibrillin 2 Rabbit pAb, Biotin conjugated(bs-12166R-Bio)-100ul
￥2980
