Description

  • Product name

    Recombinant Human AGA protein (His tag)
    See all AGA proteins and peptides
  • Purity

    > 90 % SDS-PAGE.

  • Endotoxin level

    < 1.000 Eu/µg
  • Expression system

    Baculovirus infected insect cells
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Predicted molecular weight

      36 kDa including tags
    • Amino acids

      24 to 346
    • Tags

      His tag C-Terminus
    • Additional sequence information

      This product is for the mature full length protein. The signal peptide is not included. (NP_000018)

Specifications

Our Abpromise guarantee covers the use of ab219282 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

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.4
    Constituents: 90% PBS, 10% Glycerol

General Info

  • Alternative names

    • Aga
    • AGU
    • Aspartylglucosaminidase
    • Aspartylglucosylamine deaspartylase
    • Aspartylglycosaminuria
    • ASPG_HUMAN
    • ASRG
    • GA
    • Glycosylasparaginase
    • Glycosylasparaginase beta chain
    • N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase
    • N4 (N acetyl beta glucosaminyl) L asparagine amidase
    • N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase
    see all
  • Function

    Cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins.
  • Involvement in disease

    Defects in AGA are the cause of aspartylglucosaminuria (AGU) [MIM:208400]. AGU is an inborn lysosomal storage disease. Clinical features of AGU include mild to severe mental retardation manifesting from the age of 2, coarse facial features and mild connective tissue abnormalities. This recessively inherited disease is overrepresented in the Finnish population.
  • Sequence similarities

    Belongs to the Ntn-hydrolase family.
  • Cellular localization

    Lysosome.
  • Information by UniProt

Images

  • 15% SDS-PAGE analysis of ab219282 (3μg).

References

ab219282 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab219282.
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Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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