Anti-beta glucuronidase (GUSB) 抗体 (ab88567)

製品の概要

  • 製品名Anti-beta glucuronidase (GUSB) antibody
    beta glucuronidase (GUSB) 一次抗体 製品一覧
  • 製品の詳細
    Mouse polyclonal to beta glucuronidase (GUSB)
  • 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 beta glucuronidase (GUSB) protein (NP_000172.1)

  • ポジティブ・コントロール
    • Human liver tissue lysate and beta glucuronidase (GUSB) transfected 293T cell lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab88567 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 Use a concentration of 1 µg/ml. Predicted molecular weight: 75 kDa.

ターゲット情報

  • 機能Plays an important role in the degradation of dermatan and keratan sulfates.
  • 関連疾患Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) [MIM:253220]; also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment.
    Note=Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
  • 配列類似性Belongs to the glycosyl hydrolase 2 family.
  • 翻訳後修飾N-linked glycosylated with 3 to 4 oligosaccharide chains.
  • 細胞内局在Lysosome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • Ac2-223 antibody
    • asd antibody
    • Beta G1 antibody
    • Beta glucuronidase antibody
    • Beta-G1 antibody
    • Beta-glucuronidase antibody
    • BG antibody
    • BGLR antibody
    • BGLR_HUMAN antibody
    • FLJ39445 antibody
    • Glucuronidase beta antibody
    • Gur antibody
    • Gus antibody
    • Gus-r antibody
    • Gus-s antibody
    • Gus-t antibody
    • Gus-u antibody
    • GUSB antibody
    • Gut antibody
    • MPS7 antibody
    see all

Anti-beta glucuronidase (GUSB) antibody 画像

  • Anti-beta glucuronidase (GUSB) antibody (ab88567) at 1 µg/ml + human liver tissue lysate at 50 µg

    Predicted band size : 75 kDa
    Observed band size : 60 kDa (why is the actual band size different from the predicted?)
  • All lanes : Anti-beta glucuronidase (GUSB) antibody (ab88567) at 1 µg/ml

    Lane 1 : beta glucuronidase (GUSB) transfected 293T cell lysate
    Lane 2 : Non transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 75 kDa
    Observed band size : 85 kDa (why is the actual band size different from the predicted?)

Anti-beta glucuronidase (GUSB) antibody (ab88567) 使用論文

ab88567 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"