概述

  • 产品名称Anti-PHYH抗体
    参阅全部 PHYH 一抗
  • 描述
    兔多克隆抗体to PHYH
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: 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).

  • 阳性对照
    • Human fetal brain lysate

应用

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.

应用 Ab评论 说明
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.

靶标

  • 功能Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
  • 组织特异性Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
  • 通路Lipid metabolism; fatty acid metabolism.
  • 疾病相关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.
  • 序列相似性Belongs to the PhyH family.
  • 细胞定位Peroxisome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

  • 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)参考文献

ab98131 has not yet been referenced specifically in any publications.

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