LMPD Database

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LMP000590

UniProt Annotations

Entry Information
Gene Namephytanoyl-CoA 2-hydroxylase
Protein EntryPAHX_HUMAN
UniProt IDO14832
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=O14832-1; Sequence=Displayed; Name=2; IsoId=O14832-2; Sequence=VSP_046289; Note=No experimental confirmation available. Gene prediction based on EST data.;
Catalytic ActivityPhytanoyl-CoA + 2-oxoglutarate + O(2) = 2- hydroxyphytanoyl-CoA + succinate + CO(2).
CofactorName=Fe cation; Xref=ChEBI
CofactorName=L-ascorbate; Xref=ChEBI
DiseaseRefsum disease (RD) [MIM
FunctionConverts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
InteractionQ9UNS2:COPS3; NbExp=1; IntAct=EBI-721853, EBI-350590; Q9P2S5:WDR8; NbExp=1; IntAct=EBI-721853, EBI-1054904;
PathwayLipid metabolism; fatty acid metabolism.
SimilarityBelongs to the PhyH family.
Subcellular LocationPeroxisome.
SubunitInteracts specifically with the immunophilin FKBP52 and PHYHIP. {ECO
Tissue SpecificityExpressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.