SpecificitySpecificity has been confirmed by WB and ELISA against the antigen. Human. Other species have not been tested.
Target Host SpeciesHuman
Immunogen DescriptionRecombinant human PhyH [1-338], with a GST tag.
Purity DescriptionProtein G purified immunoglobulin
FormatLyophilized from PBS pH 7.2
Reconstitution InstructionsSpin vial briefly before opening. Reconstitute in 100 µL sterile-filtered, ultrapure water. Centrifuge to remove any insoluble material.
Storage InstructionsAfter reconstitution keep aliquots at -20°C for higher stability or at 2-8°C with an appropriate antibacterial agent. Glycerol (1:1) may be added for additional stability. Avoid repetitive freeze/thaw cycles.
Batch NumberPlease see item label.
Expiration Date12 months after date of receipt (unopened vial).
PhyH is a peroxisomal protein that is involved in the alpha-oxidation of 3-methyl branched fatty acids. Specifically, this protein converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. It requires iron and ascorbate as cofactors and is expressed in liver, kidney, and T-cells but not in spleen, brain, heart, lung and skeletal muscle. Defects in PhyH are a cause of Refsum disease (RD) and deficient protein activity has been associated with Zellweger syndrome and rhizomelic chondrodysplasia punctata. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.