概述

  • 产品名称Anti-PAH抗体
    参阅全部 PAH 一抗
  • 描述
    小鼠多克隆抗体to PAH
  • 经测试应用适用于: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length Human PAH protein (AAH26251.1).

  • 阳性对照
    • WB: Human liver tissue lysate; PAH transfected 293T cell lysate IHC-P: Human kidney tissue

性能

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应用

Our Abpromise guarantee covers the use of ab88740 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB
IHC-P
  • 应用说明IHC-P: Use at a concentration of 3 µg/ml. Antigen retrieval is recommended. Place sample in 1X citrate buffer (pH 6.0) and microwave at 750W for 20 minutes, cool sample
    WB: Use at a concentration of 1 µg/ml. Detects a band of approximately 50 kDa (predicted molecular weight: 52 kDa).


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 通路Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6.
    • 疾病相关Defects in PAH are the cause of phenylketonuria (PKU) [MIM:261600]. PKU is an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol (normal concentration 100 mumol) which usually causes mental retardation (unless low phenylalanine diet is introduced early in life). They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.
      Defects in PAH are the cause of non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA) [MIM:261600]. Non-PKU HPA is a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Non-PKU HPA is usually caused by the combined effect of a mild hyperphenylalaninemia mutation and a severe one.
      Defects in PAH are the cause of hyperphenylalaninemia (HPA) [MIM:261600]. HPA is the mildest form of phenylalanine hydroxylase deficiency.
    • 序列相似性Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
      Contains 1 ACT domain.
    • Information by UniProt
    • 数据库链接
    • 别名
      • PAH antibody
      • PH antibody
      • PH4H_HUMAN antibody
      • Phe 4 monooxygenase antibody
      • Phe-4-monooxygenase antibody
      • Phenylalanine 4 hydroxylase antibody
      • Phenylalanine hydroxylase antibody
      • Phenylalanine-4-hydroxylase antibody
      • PKU antibody
      • PKU1 antibody
      see all

    Anti-PAH antibody 图像

    • Anti-PAH antibody (ab88740) at 1 µg/ml + Human liver tissue lysate at 50 µg

      Predicted band size : 52 kDa
      Observed band size : 50 kDa (why is the actual band size different from the predicted?)
      Additional bands at : <15 kDa. We are unsure as to the identity of these extra bands.
    • All lanes : Anti-PAH antibody (ab88740) at 1 µg/ml

      Lane 1 : PAH transfected 293T cell lysate
      Lane 2 : Non transfected 293T cell lysate

      Lysates/proteins at 25 µg per lane.


      Predicted band size : 52 kDa
      Observed band size : 50 kDa (why is the actual band size different from the predicted?)
    • ab88740, at 3 µg/ml, staining PAH in formalin fixed, paraffin embedded Human kidney tissue by Immunoperoxidase/ Immunohistochemistry.

    Anti-PAH antibody (ab88740)参考文献

    ab88740 has not yet been referenced specifically in any publications.

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