製品の概要

  • 製品名
    Anti-PHYH antibody
  • 製品の詳細
    Rabbit polyclonal to PHYH
  • アプリケーション
    適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat
  • 免疫原

    Recombinant fragment, corresponding to amino acids 34-321 of Human PHYH (BC029512).

  • ポジティブ・コントロール
    • Human fetal kidney and liver lysates; Human breast carcinoma tissue

法規制情報

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab118195 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 1/500 - 1/1000. Predicted molecular weight: 39 kDa.
IHC-P 1/100 - 1/500.

ターゲット情報

  • 機能
    Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
  • 組織特異性
    Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
  • パスウェイ
    Lipid metabolism; fatty acid metabolism.
  • 関連疾患
    Defects in PHYH are a cause of Refsum disease (RD) [MIM:266500]. RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life.
  • 配列類似性
    Belongs to the PhyH family.
  • 細胞内局在
    Peroxisome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • LN1 antibody
    • LNAP1 antibody
    • LNAP1, mouse, homolog of antibody
    • OTTHUMP00000019131 antibody
    • OTTHUMP00000019132 antibody
    • OTTHUMP00000179083 antibody
    • OTTHUMP00000216226 antibody
    • PAHX antibody
    • PAHX_HUMAN antibody
    • peroxisomal antibody
    • PhyH antibody
    • PHYH1 antibody
    • Phytanic acid oxidase antibody
    • phytanoil-CoA alpha hydroxylase antibody
    • phytanoyl CoA 2 hydroxylase antibody
    • Phytanoyl CoA 2 oxoglutarate dioxygenase antibody
    • Phytanoyl CoA alpha hydroxylase antibody
    • Phytanoyl CoA dioxygenase antibody
    • Phytanoyl CoA dioxygenase peroxisomal antibody
    • Phytanoyl-CoA alpha-hydroxylase antibody
    • Phytanoyl-CoA dioxygenase antibody
    • RD antibody
    see all

画像

  • All lanes : Anti-PHYH antibody (ab118195) at 1/500 dilution

    Lane 1 : Human fetal kidney lysate
    Lane 2 : Human fetal liver lysate


    Predicted band size : 39 kDa
  • ab118195, at 1/100 dilution, staining PHYH in formalin-fixed, paraffin-embedded Human breast carcinoma tissue showing cytoplasmic staining, by Immunohistochemistry.

参考文献

ab118195 has not yet been referenced specifically in any publications.

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