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MAB2642 | Anti-Fibrillin-2 Antibody, clone 48

MAB2642
100 µg  
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      Overview

      Replacement Information

      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H IHC, ELISA, IP, ICC M Purified Monoclonal Antibody
      Description
      Catalogue NumberMAB2642
      DescriptionAnti-Fibrillin-2 Antibody, clone 48
      Alternate Names
      • fibrillin 2
      • congenital contractural arachnodactyly
      • Fibrillin 5
      Background InformationFibrillin-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.
      References
      Product Information
      FormatPurified
      Control
      • Liver cancer and papillary serous carcinoma tissue
      PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Applications
      ApplicationAnti-Fibrillin-2 Antibody, clone 48 is an antibody against Fibrillin-2 for use in IH, ELISA, IP & IC.
      Key Applications
      • Immunohistochemistry
      • ELISA
      • Immunoprecipitation
      • Immunocytochemistry
      Application NotesELISA 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).
      Biological Information
      ImmunogenRecombinant protein corresponding to human Fibrillin-2.
      EpitopeUnknown
      Clone48
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityThe antibody recognizes Fibrillin-2.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThe 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].
      Gene Symbol
      • DA9
      • CCA
      • FBN2
      Purification MethodProtein G Purified
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Molecular Weight~ 215 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated 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.
      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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Fibrillin-2, clone 48 - 2120983 2120983
      Anti-Fibrillin-2, clone 48 - NG1928924 NG1928924
      Anti-Fibrillin-2, clone 48 - NRG1739024 NRG1739024
      Anti-Fibrillin-2, clone 48 -2664461 2664461
      Anti-Fibrillin-2, clone 48 -2733577 2733577

      References

      Reference overviewPub Med ID
      Fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 are strongly expressed in the epithelium of human granular and lattice type I corneal dystrophies.
      Szalai, E; Felszeghy, S; Hegyi, Z; Módis, L; Berta, A; Kaarniranta, K
      Molecular vision 18 1927-36 2012

      Show Abstract
      22876117 22876117