货号：MAB2642

品牌：Merck Millipore

规格：100Ug

目录价：￥5513.00

市场价格：￥4686.05

会员价格：￥4410.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Fibrillin-2 Antibody, clone 48 | MAB2642 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: The antibody recognizes Fibrillin-2. View All» Molecular Weight: ~ 215 kDa View All» Epitope: Unknown View All» Immunogen: Recombinant protein corresponding to human Fibrillin-2. View All» Clone: 48 View All» Isotype: IgG1κ View All» Background Information: 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. View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with human. View All» Application Notes: 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). View All» Control: Liver cancer and papillary serous carcinoma tissue View All» Quality Assurance: 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. View All» Purification Method: Protein G purfied View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: P35556 View All» Entrez Gene Number: NP_001990 View All» Gene Symbol:

• DA9

• CCA

• FBN2
  View All» Alternate Names:
  • fibrillin 2

  • congenital contractural arachnodactyly

  • Fibrillin 5
    View All» Usage Statement: 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. View All» Key Applications:
    • Immunohistochemistry

    • ELISA

    • Immunoprecipitation

    • Immunocytochemistry
      View All» Entrez Gene Summary: 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]. View All» UniProt Summary: 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. View All» Product Name: Anti-Fibrillin-2, clone 48 View All» Concentration: 1.0 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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