製品の概要

  • 製品名Anti-PAH antibody
    PAH 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to PAH
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Mouse, Human
  • 免疫原

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

  • ポジティブ・コントロール
    • Mouse liver tissue lysates

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB 1/100 - 1/500. Predicted molecular weight: 52 kDa.

ターゲット情報

  • パスウェイAmino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6.
  • 関連疾患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.
  • 配列類似性Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
    Contains 1 ACT domain.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • 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) 使用論文

ab104303 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab104303.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"