LMPD Database

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LMP002178

UniProt Annotations

Entry Information
Gene Namephytanoyl-CoA hydroxylase
Protein EntryPAHX_MOUSE
UniProt IDO35386
SpeciesMouse
Comments
Comment typeDescription
Catalytic ActivityPhytanoyl-CoA + 2-oxoglutarate + O(2) = 2- hydroxyphytanoyl-CoA + succinate + CO(2).
CofactorName=Fe cation; Xref=ChEBI:CHEBI:24875;
CofactorName=L-ascorbate; Xref=ChEBI:CHEBI:38290;
DiseaseNote=Defects in Phyh are the cause of lupus nephritis, a severe autoimmune disease. Phyh could be involved in a reaction against the progression of the disease, because its expression is low in the early stage of the disease in the renal cortex of MRL/lpr mice.
FunctionConverts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
PathwayLipid metabolism; fatty acid metabolism.
SimilarityBelongs to the PhyH family. {ECO:0000305}.
Subcellular LocationPeroxisome.
SubunitInteracts specifically with the immunophilin FKBP52 and PHYHIP. {ECO:0000269|PubMed:10686344}.
Tissue SpecificityUbiquitously expressed in all tissues with significant levels detected in the embryonic and neonatal heart and liver. In the adult, significant levels are detected in the liver, kidney, heart, gut, brain and aorta. {ECO:0000269|PubMed:10686344}.