Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP17900
    • SpeciesHuman
    • SequenceMQSLMQAPLLIALGLLLAAPAQAHLKKPSQLSSFSWDNCDEGKDPAVIRS LTLEPDPIIVPGNVTLSVMGSTSVPLSSPLKVDLVLEKEVAGLWIKIPCT DYIGSCTFEHFCDVLDMLIPTGEPCPEPLRTYGLPCHCPFKEGTYSLPKS EFVVPDLELPSWLTTGNYRIESVLSSSGKRLGCIKIAASLKGI
    • Molecular weight47 kDa including tags
    • Amino acids1 to 193

Specifications

Our Abpromise guarantee covers the use of ab116790 in the following tested applications.

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

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • Cerebroside sulfate activator protein
    • ganglioside GM2 activator
    • Ganglioside GM2 activator isoform short
    • Ganglioside GM2 activator precursor
    • GM2 activator
    • GM2 AP
    • GM2 ganglioside activator
    • GM2 ganglioside activator protein
    • GM2-AP
    • GM2A
    • GM2AP
    • OTTHUMP00000160619
    • SAP 3
    • SAP-3
    • SAP3
    • SAP3_HUMAN
    • Shingolipid activator protein 3
    • Sphingolipid activator protein 3
    see all
  • FunctionBinds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3.
  • Involvement in diseaseDefects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB) [MIM:272750]; also known as Tay-Sachs disease AB variant. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2GAB is characterized by GM2 gangliosides accumulation in the presence of both hexosaminidase A and B.
  • Post-translational
    modifications
    The serines in positions 32 and 33 are absent in 80% of the sequenced protein.
  • Cellular localizationLysosome.
  • Information by UniProt

Recombinant Human GM2A protein images

  • 12.5% SDS-PAGE showing ab116790 at approximately 47.30kDa.
    Stained with Coomassie Blue.

References for Recombinant Human GM2A protein (ab116790)

ab116790 has not yet been referenced specifically in any publications.

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