Recombinant人Fibrillin 2 protein (ab114937)

概述

  • 产品名称
    Recombinant人Fibrillin 2 protein
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      RQKRSIHEPDPTAVEQISLESVDMDSPVNMKFNLSHLGSKEHILELRPAI QPLNNHIRYVISQGNDDSVFRIHQRNGLSYLHTAKKKLMPGTYTLEITSI P
    • 分子量
      37 kDa including tags
    • 氨基酸
      2776 to 2876

技术指标

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.

  • 应用

    SDS-PAGE

    Western blot

    ELISA

  • 形式
    Liquid
  • 补充说明
    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...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • CCA
    • congenital contractural arachnodactyly (Marfanoid-like)
    • DA9
    • FBN2
    • FBN2_HUMAN
    • fibrillin 2 (congenital contractural arachnodactyly)
    • Fibrillin-2
    see all
  • 功能
    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.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the fibrillin family.
    Contains 47 EGF-like domains.
    Contains 9 TB (TGF-beta binding) domains.
  • 细胞定位
    Secreted > extracellular space > extracellular matrix.
  • Information by UniProt

图片

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

文献

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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