製品の概要

  • 製品名Anti-HEXA antibody
    HEXA 一次抗体 製品一覧
  • 製品の詳細
    Mouse polyclonal to HEXA
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Full length human HEXA protein (NP_000511.1)

  • ポジティブ・コントロール
    • HEXA transfected 293T cell lysate.

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB 1/500 - 1/1000. Detects a band of approximately 61 kDa (predicted molecular weight: 61 kDa).

ターゲット情報

  • 機能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.
  • 関連疾患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).
  • 配列類似性Belongs to the glycosyl hydrolase 20 family.
  • 翻訳後修飾N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
  • 細胞内局在Lysosome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • 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

Anti-HEXA antibody (ab70083) 使用論文

ab70083 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"