ARG21971
anti-Fibrillin 1 antibody [11C1.3] (Biotin)
anti-Fibrillin 1 antibody [11C1.3] (Biotin) for Electron microscopy,ICC/IF,IHC-Frozen sections,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Bovine
概述
产品描述 | Biotin-conjugated Mouse Monoclonal antibody [11C1.3] recognizes Fibrillin 1 |
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反应物种 | Hu, Bov |
应用 | EM, ICC/IF, IHC-Fr, IHC-P, WB |
特异性 | Human/Bovine/Japanese Macaque fibrillin-1 |
宿主 | Mouse |
克隆 | Monoclonal |
克隆号 | 11C1.3 |
同位型 | IgG1, kappa |
靶点名称 | Fibrillin 1 |
抗原物种 | Bovine |
抗原 | Microfibrils from the zonular apparatus of Bovine eye |
偶联标记 | Biotin |
別名 | ECTOL1; MFS1; WMS; SGS; SSKS; MASS; GPHYSD2; WMS2; ACMICD; OCTD; Fibrillin-1; FBN |
应用说明
应用建议 |
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应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
属性
形式 | Liquid |
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缓冲液 | PBS and 0.1% Sodium azide. |
抗菌剂 | 0.1% Sodium azide |
浓度 | 0.2 mg/ml |
存放说明 | Aliquot and store in the dark at 2-8°C. Keep protected from prolonged exposure to light. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
数据库连接 | |
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基因名称 | FBN1 |
全名 | fibrillin 1 |
背景介绍 | This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. [provided by RefSeq, Jul 2008] |
生物功能 | 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-1-containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. [UniProt] |
预测分子量 | 312 kDa |
翻译后修饰 | Fibrillin-1: Cleavage of N- and C-terminus by furin is required for incorporation into the extracellular matrix and assembly into microfibrils (PubMed:27026396). The C-terminus, which corresponds to the Asprosin chain, was initially thought to constitute a propeptide (PubMed:24982166). Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin, an essential step for incorporation of Fibrillin-1 into the nascent microfibrils (PubMed:24982166). Fibrillin-1: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils. |