Recombinant人FANCB protein (ab116811)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionQ8NB91
    • 种属Human
    • 序列GSENFLIDNMAFTLEKELVTLSSLSSAIAKHESNFMQRCEVSKGKSSVVA AALSDRRENIHPYRKELQREKKKMLQTNLKVSGALYREITLKVAEVQLKS DFAAQKLSN
    • 分子量38 kDa including tags
    • 氨基酸750 to 858

技术指标

Our Abpromise guarantee covers the use of ab116811 in the following tested applications.

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

  • 应用

    Western blot

    ELISA

    SDS-PAGE

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    This protein is best used within three months from the date of receipt.
  • 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: 0.79% Tris HCl, 0.3% Glutathione

常规信息

  • 别名
    • FA2
    • FAAP90
    • FAAP95
    • FAB
    • FACB
    • Fancb
    • FANCB_HUMAN
    • Fanconi anemia associated polypeptide of 95 kDa
    • Fanconi anemia group B protein
    • Fanconi anemia, complementation group B
    • Fanconi anemia-associated polypeptide of 95 kDa
    • Protein FACB
    see all
  • 功能DNA repair protein required for FANCD2 ubiquitination.
  • 疾病相关Defects in FANCB are the cause of Fanconi anemia complementation group B (FANCB) [MIM:300514]. It is a disorder affecting all bone marrow elements and resulting in anemia, leukopenia and thrombopenia. It is associated with cardiac, renal and limb malformations, dermal pigmentary changes, and a predisposition to the development of malignancies. At the cellular level it is associated with hypersensitivity to DNA-damaging agents, chromosomal instability (increased chromosome breakage) and defective DNA repair.
    Defects in FANCB are the cause of X-linked VACTERL-H (XVACTERL-H) [MIM:314390]; also known as X-linked VACTERL association with hydrocephalus syndrome. VACTERL is an acronym for vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies (urethral atresia with hydronephrosis), and limb anomalies (hexadactyly, humeral hypoplasia, radial aplasia, and proximally placed thumb). Some cases of VACTERL-H are associated with increased chromosome breakage and rearrangement.
  • 细胞定位Nucleus.
  • Information by UniProt

Recombinant Human FANCB protein 图像

  • 12.5% SDS-PAGE showing ab116811 at approximately 37.62kDa.
    Stained with Coomassie Blue.

Recombinant Human FANCB protein (ab116811)参考文献

ab116811 has not yet been referenced specifically in any publications.

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