概述

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

    Synthetic peptide corresponding to a region within internal sequence amino acids 140-170 of Human PAH, conjugated to KLH.

  • 阳性对照
    • Mouse liver tissue lysates

性能

应用

Our Abpromise guarantee covers the use of ab104303 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 1/100 - 1/500. Predicted molecular weight: 52 kDa.

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 (ab104303) at 1/100 dilution + Mouse liver tissue lysates at 35 µg

    Predicted band size : 52 kDa
  • Anti-PAH antibody (ab104303) at 1/500 dilution + Recombinant Human PAH protein (ab88738) at 0.01 µg

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Exposure time : 4 minutes

Anti-PAH antibody (ab104303)参考文献

ab104303 has not yet been referenced specifically in any publications.

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