Overview

  • Product name

  • Description

    Rabbit polyclonal to Fibrillin 2
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to Human Fibrillin 2 aa 364-493.
    Sequence:

    GMCFSGLVNGRCAQELPGRMTKMQCCCEPGRCWGIGTIPEACPVRGSEEY RRLCMDGLPMGGIPGSAGSRPGGTGGNGFAPSGNGNGYGPGGTGFIPIPG GNGFSPGVGGAGVGAGGQGPIITGLTILNQ


    Database link: P35556

  • Positive control

    • IHC: Human placenta tissue. WB: U-2 OS cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab224256 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
IHC-P 1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
WB 1/100 - 1/250. Predicted molecular weight: 315 kDa.

Target

  • 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. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.
  • 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.
  • Cellular localization

    Secreted > extracellular space > extracellular matrix.
  • Information by UniProt
  • Database links

  • Alternative names

    • CCA antibody
    • congenital contractural arachnodactyly (Marfanoid-like) antibody
    • DA9 antibody
    • FBN2 antibody
    • FBN2_HUMAN antibody
    • fibrillin 2 (congenital contractural arachnodactyly) antibody
    • Fibrillin-2 antibody
    see all

Images

  • Paraffin-embedded human placenta tissue stained for Fibrillin 2 using ab224256 at 1/50 dilution in immunohistochemical analysis.

  • Anti-Fibrillin 2 antibody (ab224256) at 1/100 dilution + U-2 OS (human bone osteosarcoma epithelial cell line) cell lysate

    Developed using the ECL technique.

    Predicted band size: 315 kDa

References

ab224256 has not yet been referenced specifically in any publications.

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