Overview

  • Product nameAnti-PHYH antibody
    See all PHYH primary antibodies
  • Description
    Rabbit polyclonal to PHYH
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Dog
  • Immunogen

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

  • Positive control
    • Human fetal brain lysate

Applications

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.

Application Abreviews Notes
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.

Target

  • FunctionConverts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
  • Tissue specificityExpressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
  • PathwayLipid metabolism; fatty acid metabolism.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the PhyH family.
  • Cellular localizationPeroxisome.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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 images

  • 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

References for Anti-PHYH antibody (ab98131)

ab98131 has not yet been referenced specifically in any publications.

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