- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H ICC, IHC, ELISA M Purified Monoclonal Antibody 描述 产品目录编号 MAB2642 描述 Anti-Fibrillin-2 Antibody, clone 48 Alternate Names - Fibrillin 5
- fibrillin 2
- congenital contractural arachnodactyly
背景信息 Fibrillin-2 is a part of the Fibrillin family of glycoproteins, which are essential factors in the formation and maintenance of elastic fibers known as microfibrils within the extracellular matrix. Various researchers have suggested that Fibrillin-2 has a role in the mediation of microfibril assembly during early elastogenesis. Defects in Fibrillin-2 are causal to a rare disorder involving autosomal dominant connective tissue known as congenital contractual arachnodactyly (CCA), also known as Beals syndrome or distal arthrogryposis type 9 (DA9), and is similar to Marfan syndrome. 产品信息 格式 Purified 控制 - Liver cancer and papillary serous carcinoma tissue
演示 Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Anti-Fibrillin-2 Antibody, clone 48 is an antibody against Fibrillin-2 for use in IH, ELISA, IP & IC. 主要应用 - Immunocytochemistry
- Immunohistochemistry
- ELISA
应用说明 ELISA Analysis: A previous lot was used by an independent laboratory in ELISA (Charbonneau, 2003).
Immunofluorescence Analysis: A previous lot was used by an independent laboratory in IF (Charbonneau, 2003).生物信息 免疫原品种 Recombinant protein corresponding to human Fibrillin-2. 表位 Unknown 克隆 48 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 The antibody recognizes Fibrillin-2. 同种型 IgG1κ 品种反应性 Human Species Reactivity Note Demonstrated to react with human. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_001990
Entrez基因汇总 The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq]. 基因符号 - FBN2
- DA9
- CCA
纯化方法 Protein G purfied UniProt编号 - P35556
UniProt汇总 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.
SUBCELLULAR LOCATION: Secreted › extracellular space › extracellular matrix.
INVOLVEMENT IN 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.
SEQUENCE SIMILARITIES: Belongs to the fibrillin family.
Contains 47 EGF-like domains.
Contains 9 TB (TGF-beta binding) domains.产品使用声明 质量保证 Evaluated by Immunohistochemistry in liver cancer and papillary serous carcinoma paraffin-embedded tissue sections.
Immunohistochemistry Analysis: 1:50 dilution of this antibody detected Fibrillin-2 in paraffin-embedded tissue sections of liver cancer and papillary serous carcinoma.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
上一件merck millipore产品：merck millipore,默克密理博,EZGRT-89K,Human Ghrelin (total) ELISA
下一件merck millipore产品：merck millipore,默克密理博,506157,p38 MAP Kinase Inhibitor VI, JX401 - CAS 349087-34-9 - Calbiochem
相关产品
merck millipore,默克密理博,71012,HT96™ BL21(DE3) Competent Cells - Novagen
merck millipore,默克密理博,189482,Autocamtide-2 Related Inhibitory Peptide, Myristoylated - Calbiochem
merck millipore,默克密理博,EZGRT-89K,Human Ghrelin (total) ELISA
merck millipore,默克密理博,506157,p38 MAP Kinase Inhibitor VI, JX401 - CAS 349087-34-9 - Calbiochem
merck millipore,默克密理博,ABN467,Anti-CX3CL1 Antibody
merck millipore,默克密理博,MABN263,Anti-GABA(A) receptor subunit gamma-2 Antibody, clone 10F10-C1-B8
merck millipore,默克密理博,9285-OP,OmniPur TRIS Solution, 1.0M pH 7.5 - Calbiochem
merck millipore,默克密理博,324405,Anti-Dopamine D₄ Receptor Rabbit pAb