製品の概要

  • 製品名Anti-Galactosidase alpha antibody
    Galactosidase alpha 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Galactosidase alpha
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Non Human Primates
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Galactosidase alpha.

    (Peptide available as ab91202.)

  • ポジティブ・コントロール
    • This antibody gave a positive signal in Human Liver Tissue Lysate.

製品の特性

  • 製品の状態Liquid
  • 保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • バッファーPreservative: 0.02% Sodium Azide
    Constituents: 1% BSA, PBS, pH 7.4
  • Concentration information loading...
  • 精製度Immunogen affinity purified
  • ポリ/モノポリクローナル
  • アイソタイプIgG
  • 研究分野

アプリケーション

Our Abpromise guarantee covers the use of ab70520 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. Detects a band of approximately 49 kDa (predicted molecular weight: 49 kDa).

ターゲット情報

  • 関連疾患Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
  • 配列類似性Belongs to the glycosyl hydrolase 27 family.
  • 細胞内局在Lysosome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • AGAL_HUMAN antibody
    • Agalsidase alfa antibody
    • Alpha D galactosidase A antibody
    • Alpha D galactoside galactohydrolase 1 antibody
    • Alpha D galactoside galactohydrolase antibody
    • Alpha gal A antibody
    • Alpha galactosidase A antibody
    • Alpha-D-galactosidase A antibody
    • Alpha-D-galactoside galactohydrolase antibody
    • Alpha-galactosidase A antibody
    • GALA antibody
    • Galactosidase, alpha antibody
    • GLA antibody
    • GLA protein antibody
    • Melibiase antibody
    see all

Anti-Galactosidase alpha antibody 画像

  • Anti-Galactosidase alpha antibody (ab70520) at 1 µg/ml + Human liver tissue lysate - total protein (ab29889) at 10 µg

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 49 kDa
    Observed band size : 49 kDa
    Additional bands at : 47 kDa (possible cleavage fragment).

    Galactosidase alpha contains a 31 amino acid signal peptide. We hypothesize that the 47 kDa band represents the mature protein. Some batches of this product also recognise a band at 100 kDa in Western Blot, we are unsure of the identity of this band.

Anti-Galactosidase alpha antibody (ab70520) 使用論文

This product has been referenced in:
  • Andreotti G  et al. A thermodynamic assay to test pharmacological chaperones for Fabry disease. Biochim Biophys Acta 1840:1214-24 (2014). WB . Read more (PubMed: 24361605) »

See 1 Publication for this product

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