Recombinant Human Fibrillin 2 protein (ab114937)

Overview

  • Product name
    Recombinant Human Fibrillin 2 protein
  • Protein length
    Protein fragment

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      RQKRSIHEPDPTAVEQISLESVDMDSPVNMKFNLSHLGSKEHILELRPAI QPLNNHIRYVISQGNDDSVFRIHQRNGLSYLHTAKKKLMPGTYTLEITSI P
    • Molecular weight
      37 kDa including tags
    • Amino acids
      2776 to 2876

Specifications

Our Abpromise guarantee covers the use of ab114937 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
    Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

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

Images

  • ab114937 on a 12.5% SDS-PAGE Stained with Coomassie Blue.

References

ab114937 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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