概述

  • 产品名称
  • 描述
    小鼠多克隆抗体to HEXA
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length human HEXA protein (NP_000511.1)

  • 阳性对照
    • HEXA transfected 293T cell lysate.

性能

应用

Our Abpromise guarantee covers the use of ab70083 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 1/500 - 1/1000. Detects a band of approximately 61 kDa (predicted molecular weight: 61 kDa).

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

图片

  • All lanes : Anti-HEXA antibody (ab70083) at 1/500 dilution

    Lane 1 : HEXA transfected 293T cell lysate
    Lane 2 : Non-transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 61 kDa
    Observed band size : 61 kDa

实验方案

文献

ab70083 has not yet been referenced specifically in any publications.

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