Recombinant人FUCA1 protein (ab114799)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP04066
    • 种属Human
    • 序列EAIYASKPWRVQWEKNTTSVWYTSKGSAVYAIFLHWPENGVLNLESPITT STTKITMLGIQGDLKWSTDPDKGLFISLPQLPPSAVPAEFAWTIKLTGVK
    • 分子量37 kDa including tags
    • 氨基酸367 to 466

技术指标

Our Abpromise guarantee covers the use of ab114799 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

    SDS-PAGE

    ELISA

  • 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: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • Alpha L fucosidase I
    • Alpha L fucoside fucohydrolase
    • Alpha-L-fucosidase 1
    • Alpha-L-fucosidase I
    • Alpha-L-fucoside fucohydrolase 1
    • FUCA
    • FUCA1
    • FUCO_HUMAN
    • fucosidase, alpha-L, tissue
    • fucosidase, alpha-L- 1, tissue
    • Tissue alpha L fucosidase
    • Tissue alpha-L-fucosidase
    see all
  • 功能Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins.
  • 疾病相关Defects in FUCA1 are the cause of fucosidosis (FUCA1D) [MIM:230000]. FUCA1D is an autosomal recessive lysosomal storage disease characterized by accumulation of fucose-containing glycolipids and glycoproteins in various tissues. Clinical signs include facial dysmorphism, dysostosis multiplex, moderate hepatomegaly, severe intellectual deficit, deafness, and according to age, angiokeratomas.
  • 序列相似性Belongs to the glycosyl hydrolase 29 family.
  • 细胞定位Lysosome.
  • Information by UniProt

Recombinant Human FUCA1 protein 图像

  • ab114799 on 12.5% SDS-PAGE Stained with Coomassie Blue.

Recombinant Human FUCA1 protein (ab114799)参考文献

ab114799 has not yet been referenced specifically in any publications.

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