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
    Goat 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, Rat, Human
    交差が予測される動物種: Chicken, Cow, Dog
  • 免疫原

    Synthetic peptide:

    ESRPSRLKKDE

    by a Cysteine residue linker, corresponding to internal sequence amino acids 66-76 of Human PAH (NP_000268.1).

  • ポジティブ・コントロール
    • Human Liver lysates

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab106805 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 0.3 - 1 µg/ml. Detects a band of approximately 48 kDa (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

  • All lanes : Anti-PAH antibody (ab106805) at 0.10000000149 µg/ml

    Lane 1 : Mouse Liver lysate in RIPA buffer
    Lane 2 : Rat Liver lysate in RIPA buffer

    Lysates/proteins at 35 µg per lane.

    Developed using the ECL technique

    Predicted band size : 52 kDa
  • Anti-PAH antibody (ab106805) at 0.3 µg/ml + Human Liver lysate (in RIPA buffer) at 35 µg
    Developed using the ECL technique

    Predicted band size : 52 kDa
    Observed band size : 48 kDa (why is the actual band size different from the predicted?)

Anti-PAH antibody (ab106805) 使用論文

ab106805 has not yet been referenced specifically in any publications.

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