Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MEQLRAAARLQIVLGHLGRPSAGAVVAHPTSGTISSASFHPQQFQYTLDN NVLTLEQRKFYEENGFLVIKNLVPDADIQRFRNEFEKICRKEVKPLGLTV MRDVTISKSEYAPSEKMITKVQDFQEDKELFRYCTLPEILKYVECFTGPN IMAMHTMLINKPPDSGKKTSRHPLHQDLHYFPFRPSDLIVCAWTAMEHIS RNNGCLVVLPGTHKGSLKPHDYPKWEGGVNKMFHGIQDYEENKARVHLVM EKGDTVFFHPLLIHGSGQNKTQGFRKAISCHFASADCHYIDVKGTSQENI EKEVVGIAHKFFGAENSVNLKDIWMFRARLVKGERTNL
    • Molecular weight

      63 kDa including tags
    • Amino acids

      1 to 338

Associated products

Specifications

Our Abpromise guarantee covers the use of ab132583 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • LN1
    • LNAP1
    • LNAP1, mouse, homolog of
    • OTTHUMP00000019131
    • OTTHUMP00000019132
    • OTTHUMP00000179083
    • OTTHUMP00000216226
    • PAHX
    • PAHX_HUMAN
    • peroxisomal
    • PhyH
    • PHYH1
    • Phytanic acid oxidase
    • phytanoil-CoA alpha hydroxylase
    • phytanoyl CoA 2 hydroxylase
    • Phytanoyl CoA 2 oxoglutarate dioxygenase
    • Phytanoyl CoA alpha hydroxylase
    • Phytanoyl CoA dioxygenase
    • Phytanoyl CoA dioxygenase peroxisomal
    • Phytanoyl-CoA alpha-hydroxylase
    • Phytanoyl-CoA dioxygenase
    • RD
    see all
  • Function

    Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
  • Tissue specificity

    Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
  • Pathway

    Lipid metabolism; fatty acid metabolism.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the PhyH family.
  • Cellular localization

    Peroxisome.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE analysis of ab132583 stained with Coomassie Blue.

References

ab132583 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab132583.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up