Recombinant Human HEXB protein (ab198424)

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Description

  • Product name

    Recombinant Human HEXB protein

  • Purity

    > 82 % SDS-PAGE.

  • Expression system

    Baculovirus infected Sf9 cells

  • Accession

    P07686

  • Protein length

    Full length protein

  • Animal free

    No

  • Nature

    Recombinant

    • Species

      Human

    • Sequence

      MELCGLGLPRPPMLLALLLATLLAAMLALLTQVALVVQVAEAARAPSVSA KPGPALWP LPLSVKMTPNLLHLAPENFYISHSPNSTAGPSCTLLEEAF RRYHGYIFGFYKWHHEPAE FQAKTQVQQLLVSITLQSECDAFPNISSD ESYTLLVKEPVAVLKANRVWGALRGLETFS QLVYQDSYGTFTINESTI IDSPRFSHRGILIDTSRHYLPVKIILKTLDAMAFNKFNVLHWHIV DDQ SFPYQSITFPELSNKGSYSLSHVYTPNDVRMVIEYARLRGIRVLPEFDTP GHTLSWG KGQKDLLTPCYSRQNKLDSFGPINPTLNTTYSFLTTFFKEI SEVFPDQFIHLGGDEVEFK CWESNPKIQDFMRQKGFGTDFKKLESFYI QKVLDIIATINKGSIVWQEVFDDKAKLAPGTI VEVWKDSAYPEELSRV TASGFPVILSAPWYLDLISYGQDWRKYYKVEPLDFGGTQKQK QLFIGG EACLWGEYVDATNLTPRLWPRASAVGERLWSSKDVRDMDDAYDRLTRHRC R MVERGIAAQPLYAGYCNHENMDYKDDDDKHHHHHH

    • Predicted molecular weight

      65 kDa including tags

    • Tags

      DDDDK tag C-Terminus

Specifications

Our Abpromise guarantee covers the use of ab198424 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 on Dry Ice. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.0
    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 beta chain
    • Beta hexosaminidase subunit beta
    • Beta N acetylhexosaminidase
    • Beta-hexosaminidase subunit beta chain A
    • Beta-N-acetylhexosaminidase subunit beta
    • Cervical cancer proto oncogene 7 protein
    • Cervical cancer proto-oncogene 7 protein
    • ENC 1AS
    • Epididymis luminal protein 248
    • HCC 7
    • HCC-7
    • HCC7
    • HEL 248
    • HEX B
    • Hexb
    • HEXB_HUMAN
    • Hexosaminidase B
    • Hexosaminidase B (beta polypeptide)
    • Hexosaminidase subunit B
    • HexosaminidaseB
    • N acetyl beta glucosaminidase
    • N-acetyl-beta-glucosaminidase subunit beta
    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.

  • Involvement in disease

    Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2) [MIM:268800]; also known as Sandhoff disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula.

  • Sequence similarities

    Belongs to the glycosyl hydrolase 20 family.

  • Post-translational
    modifications

    N-linked glycans at Asn-142 and Asn-190 consist of Man(3)-GlcNAc(2) and Man(5 to 7)-GlcNAc(2), respectively.
    The beta-A and beta-B chains are produced by proteolytic processing of the precursor beta chain.

  • Cellular localization

    Lysosome.
  • Information by UniProt

Images

  • SDS-PAGE - Recombinant Human HEXB protein (ab198424)
    SDS-PAGE - Recombinant Human HEXB protein (ab198424)

    4-20% SDS-PAGE Coomassie staining.

    Lane 1: 2.8 µg ab198424

    Lane 2: Protein Marker

References

ab198424 has not yet been referenced specifically in any publications.

Publishing research using ab198424? Please let us know so that we can cite the reference in this datasheet.

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