概述

  • 产品名称Anti-HEXA抗体- N-terminal
    参阅全部 HEXA 一抗
  • 描述
    兔多克隆抗体to HEXA - N-terminal
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

  • 阳性对照
    • HepG2 cell lysate

性能

应用

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.

应用 Ab评论 说明
WB 1/100 - 1/500. Predicted molecular weight: 61 kDa.
ELISA 1/1000.

靶标

  • 功能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.
  • 疾病相关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).
  • 序列相似性Belongs to the glycosyl hydrolase 20 family.
  • 翻译后修饰N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
  • 细胞定位Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

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

    Predicted band size : 61 kDa

Anti-HEXA antibody - N-terminal (ab91624)参考文献

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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