Recombinant人GM2A protein (ab116790)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP17900
    • 种属Human
    • 序列MQSLMQAPLLIALGLLLAAPAQAHLKKPSQLSSFSWDNCDEGKDPAVIRS LTLEPDPIIVPGNVTLSVMGSTSVPLSSPLKVDLVLEKEVAGLWIKIPCT DYIGSCTFEHFCDVLDMLIPTGEPCPEPLRTYGLPCHCPFKEGTYSLPKS EFVVPDLELPSWLTTGNYRIESVLSSSGKRLGCIKIAASLKGI
    • 分子量47 kDa including tags
    • 氨基酸1 to 193

技术指标

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.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能Binds 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.
  • 疾病相关Defects 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.
  • 翻译后修饰The serines in positions 32 and 33 are absent in 80% of the sequenced protein.
  • 细胞定位Lysosome.
  • Information by UniProt

Recombinant Human GM2A protein 图像

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

Recombinant Human GM2A protein (ab116790)参考文献

ab116790 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab116790.
Please use the links above to contact us or submit feedback about this product.

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