Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      EAIYASKPWRVQWEKNTTSVWYTSKGSAVYAIFLHWPENGVLNLESPITT STTKITMLGIQGDLKWSTDPDKGLFISLPQLPPSAVPAEFAWTIKLTGVK
    • Molecular weight
      37 kDa including tags
    • Amino acids
      367 to 466

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • Form
    Liquid
  • Additional notes
    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...

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
    • 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
  • Function
    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.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the glycosyl hydrolase 29 family.
  • Cellular localization
    Lysosome.
  • Information by UniProt

Images

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

References

ab114799 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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