Recombinant Human AGA protein (denatured) (ab202173)

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

    Key features and details

    • Expression system: Escherichia coli
    • Purity: > 90% SDS-PAGE
    • Tags: His tag N-Terminus
    • Suitable for: SDS-PAGE

    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: 8.00
      Constituents: 0.32% Tris HCl, 10% Glycerol (glycerin, glycerine)

    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).

    Protocols

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    References (0)

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

    ab202173 has not yet been referenced specifically in any publications.

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