Overview

  • Product nameAnti-HEXA antibody - N-terminal
    See all HEXA primary antibodies
  • Description
    Rabbit polyclonal to HEXA - N-terminal
  • Tested applicationsSuitable for: WB, ELISAmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide conjugated to KLH selected from the N terminal region of Human HEXA.

  • Positive control
    • HepG2 cell lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab91624 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/100 - 1/500. Predicted molecular weight: 61 kDa.
ELISA 1/1000.

Target

  • FunctionResponsible 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 diseaseDefects 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 similaritiesBelongs to the glycosyl hydrolase 20 family.
  • Post-translational
    modifications
    N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
  • Cellular localizationLysosome.
  • Information by UniProt
  • Database links
  • Alternative names
    • Beta hexosaminidase alpha chain precursor antibody
    • Beta hexosaminidase subunit alpha antibody
    • Beta N acetylhexosaminidase antibody
    • Beta N acetylhexosaminidase subunit alpha antibody
    • Beta-hexosaminidase A antibody
    • Beta-hexosaminidase subunit alpha antibody
    • Beta-N-acetylhexosaminidase subunit alpha antibody
    • Hexa antibody
    • HEXA_HUMAN antibody
    • Hexosaminidase A (alpha polypeptide) antibody
    • Hexosaminidase A alpha polypeptide antibody
    • Hexosaminidase A antibody
    • Hexosaminidase subunit A antibody
    • MGC99608 antibody
    • N acetyl beta glucosaminidase antibody
    • N acetyl beta glucosaminidase subunit alpha antibody
    • N-acetyl-beta-glucosaminidase subunit alpha antibody
    • TSD antibody
    see all

Anti-HEXA antibody - N-terminal images

  • Anti-HEXA antibody - N-terminal (ab91624) at 1/100 dilution + HepG2 cell lysate at 35 µg

    Predicted band size : 61 kDa

References for Anti-HEXA antibody - N-terminal (ab91624)

This product has been referenced in:
  • Yasui N  et al. Molecular pathology of Sandhoff disease with p.Arg505Gln in HEXB: application of simulation analysis. J Hum Genet 58:611-7 (2013). Read more (PubMed: 23759947) »

See 1 Publication for this product

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"