Description

  • Product name

    Recombinant Human HEXA protein
    See all HEXA proteins and peptides
  • Purity

    >= 91 % SDS-PAGE.

  • Expression system

    Baculovirus infected Sf9 cells
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MTSSRLWFSLLLAAAFAGRATALWPWPQNFQTSDQRYVLYPNNFQFQYDV S SAAQPGCSVLDEAFQRYRDLLFGSGSWPRPYLTGKRHTLEKNVLVVS VVTPGCNQLPTLESVENYTLTINDDQCLLLSETVWGALRGLETFSQLVWK SAEGTFFINKTEIEDFPRFPHRGLLLDTSRHYLPLSSILDTLDVMAYNKL NVFHWHLVDDPSFPYESFTFPELMRKGSYNPVTHIYTAQDVKEVIEYARL RGIRVLAEFDTPGHTLSWGPGIPGLLTPCYSGSEPSGTFGPVNPSLNNTY EFMSTFFLEVSSVFPDFYLHLGGDEVDFTCWKSNPEIQDFMRKKGFGEDF KQLESFYIQTLLDIVSSYGKGYVVWQEVFDNKVKIQPDTIIQVWREDIPV NYMKELELVTKAGFRALLSAPWYLNRISYGPDWKDFYIVEPLAFEGTPEQ KALVIGGEACMWGEYVDNTNLVPRLWPRAGAVAERLWSNKLTSDLTFAYE RLSHFRCELLRRGVQAQPLNVGFCEQEFEQTDYKDDDDKHHHHHH
    • Predicted molecular weight

      63 kDa including tags
    • Amino acids

      1 to 529
    • Tags

      His-DDDDK tag C-Terminus

Specifications

Our Abpromise guarantee covers the use of ab198482 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
  • Additional notes

    ab198482 is useful for the study of enzyme kinetics, screening inhibitors, and selectivity profiling.

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on Dry Ice. Store at -80°C. Avoid freeze / thaw cycle.

    pH: 8
    Preservative: 1.02% Imidazole
    Constituents: 0.63% Tris HCl, 0.64% Sodium chloride, 0.02% Potassium chloride, 0.04% Tween, 20% Glycerol

General Info

  • Alternative names

    • Beta hexosaminidase alpha chain precursor
    • Beta hexosaminidase subunit alpha
    • Beta N acetylhexosaminidase
    • Beta N acetylhexosaminidase subunit alpha
    • Beta-hexosaminidase A
    • Beta-hexosaminidase subunit alpha
    • Beta-N-acetylhexosaminidase subunit alpha
    • Hexa
    • HEXA_HUMAN
    • Hexosaminidase A
    • Hexosaminidase A (alpha polypeptide)
    • Hexosaminidase A alpha polypeptide
    • Hexosaminidase subunit A
    • MGC99608
    • N acetyl beta glucosaminidase
    • N acetyl beta glucosaminidase subunit alpha
    • N-acetyl-beta-glucosaminidase subunit alpha
    • TSD
    see all
  • Function

    Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
  • Involvement in disease

    Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1) [MIM:272800]; also known as Tay-Sachs disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G1 is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. GM2G1 has an increased incidence among Ashkenazi Jews and French Canadians in eastern Quebec. It exists in several forms: infantile (most common and most severe), juvenile and adult (late onset).
  • Sequence similarities

    Belongs to the glycosyl hydrolase 20 family.
  • Post-translational
    modifications

    N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
  • Cellular localization

    Lysosome.
  • Information by UniProt

Images

  • 4-20% SDS PAGE analysis of ab198482 (2.8 μg).

References

ab198482 has not yet been referenced specifically in any publications.

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