製品の概要

  • 製品名Anti-PHYH antibody
    PHYH 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to PHYH
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Dog
  • 免疫原

    Synthetic peptide corresponding to a region within internal amino acids 251-300 (EKGDTVFFHP LLIHGSGQNK TQGFRKAISC HFASADCHYI DVKGTSQENI) of Human PHYH (NP_006205).

  • ポジティブ・コントロール
    • Human fetal brain lysate

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab98131 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 1 µg/ml. Predicted molecular weight: 38 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

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

Anti-PHYH antibody 画像

  • Anti-PHYH antibody (ab98131) at 1 µg/ml (in 5% skim milk / PBS buffer) + Human fetal brain lysate at 10 µg

    Predicted band size : 38 kDa

Anti-PHYH antibody (ab98131) 使用論文

ab98131 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab98131.
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"