Recombinant Human AGA protein (denatured) (ab202173)

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Description

  • Product name

    Recombinant Human AGA protein (denatured)
    See all AGA proteins and peptides

  • Purity

    > 90 % SDS-PAGE.

  • Expression system

    Escherichia coli

  • Accession

    P20933

  • Protein length

    Full length protein

  • Animal free

    No

  • Nature

    Recombinant

    • Species

      Human

    • Sequence

      MGSSHHHHHHSSGLVPRGSHMGSSSPLPLVVNTWPFKNATEAAWRALASG GSALDAVESGCAMCEREQCDGSVGFGGSPDELGETTLDAMIMDGTTMDVG AVGDLRRIKNAIGVARKVLEHTTHTLLVGESATTFAQSMGFINEDLSTTA SQALHSDWLARNCQPNYWRNVIPDPSKYCGPYKPPGILKQDIPIHKETED DRGHDTIGMVVIHKTGHIAAGTSTNGIKFKIHGRVGDSPIPGAGAYADDT AGAAAATGNGDILMRFLPSYQAVEYMRRGEDPTIACQKVISRIQKHFPEF FGAVICANVTGSYGAACNKLSTFTQFSFMVYNSEKNQPTEEKVDCI

    • Predicted molecular weight

      37 kDa

    • Amino acids

      24 to 346

    • Tags

      His tag N-Terminus

    • Additional sequence information

      Alpha and beta chains without signal peptide. NP_000018.

Specifications

Our Abpromise guarantee covers the use of ab202173 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: 8
    Constituents: 0.32% Tris HCl, 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

  • SDS-PAGE - Recombinant Human AGA protein (denatured) (ab202173)
    SDS-PAGE - Recombinant Human AGA protein (denatured) (ab202173)

    15% SDS-PAGE of ab202173 (3 µg).

References

ab202173 has not yet been referenced specifically in any publications.

Publishing research using ab202173? Please let us know so that we can cite the reference in this datasheet.

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