Anti-Iduronate 2 sulfatase 抗体 (ab70025)

製品の概要

  • 製品名
    Anti-Iduronate 2 sulfatase antibody
    Iduronate 2 sulfatase 一次抗体 製品一覧
  • 製品の詳細
    Mouse polyclonal to Iduronate 2 sulfatase
  • 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 Iduronate 2 sulfatase (NP_000193.1, aa 1 - 550)

  • ポジティブ・コントロール
    • Iduronate 2 sulfatase transfected 293T lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab70025 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: 62 kDa).

ターゲット情報

  • 機能
    Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.
  • 組織特異性
    Liver, kidney, lung, and placenta.
  • 関連疾患
    Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2) [MIM:309900]; also known as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation.
  • 配列類似性
    Belongs to the sulfatase family.
  • 翻訳後修飾
    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
    • Alpha L iduronate sulfate sulfatase antibody
    • Alpha-L-iduronate sulfate sulfatase antibody
    • AW214631 antibody
    • Ids antibody
    • IDS_HUMAN antibody
    • Iduronate 2 sulfatase 14 kDa chain antibody
    • Iduronate 2 sulfatase 42 kDa chain antibody
    • Iduronate 2 sulfatase antibody
    • Iduronate 2-sulfatase 14 kDa chain antibody
    • Iduronate sulfatase antibody
    • Idursulfase antibody
    • MPS2 antibody
    • RP23-29M4.1 antibody
    • SIDS antibody
    see all

画像

  • All lanes : Anti-Iduronate 2 sulfatase antibody (ab70025) at 1/500 dilution

    Lane 1 : Iduronate 2 sulfatase 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 : 62 kDa
    Observed band size : 75 kDa (why is the actual band size different from the predicted?)

プロトコール

参考文献

ab70025 has not yet been referenced specifically in any publications.

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