製品の概要

  • 製品名Anti-GNS antibody
    GNS 一次抗体 製品一覧
  • 製品の詳細
    Mouse polyclonal to GNS
  • 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 GNS (NP_002067.1).

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

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab69971 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 75 kDa (predicted molecular weight: 63 kDa).

ターゲット情報

  • 関連疾患Defects in GNS are the cause of mucopolysaccharidosis type 3D (MPS3D) [MIM:252940]; also known as Sanfilippo D syndrome. MPS3D is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life.
  • 配列類似性Belongs to the sulfatase family.
  • 翻訳後修飾The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species.
    The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
  • 細胞内局在Lysosome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 2610016K11Rik antibody
    • AU042285 antibody
    • C87209 antibody
    • G6S antibody
    • Glucosamine (N-acetyl) 6 sulfatase antibody
    • Glucosamine 6 sulfatase antibody
    • Glucosamine-6-sulfatase antibody
    • GNS antibody
    • GNS_HUMAN antibody
    • MGC21274 antibody
    • N acetylglucosamine 6 sulfatase [Precursor] antibody
    • N-acetylglucosamine-6-sulfatase antibody
    • N28088 antibody
    see all

Anti-GNS antibody 画像

  • All lanes : Anti-GNS antibody (ab69971) at 1/500 dilution

    Lane 1 : GNS transfected 293T lysate
    Lane 2 : Non transfected 293T lysate

    Lysates/proteins at 25 µg per lane.

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

    Predicted band size : 63 kDa
    Observed band size : 75 kDa (why is the actual band size different from the predicted?)

Anti-GNS antibody (ab69971) 使用論文

ab69971 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab69971.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"