phytanoyl-CoA dioxygenase, peroxisomal; phytanic acid oxidase; phytanoyl-CoA alpha-hydroxylase; phyH (PHYH, PAHX)

Function

• converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA
• lipid metabolism; fatty acid metabolism
• interacts specifically with the immunophilin FKBP52 & PHYHIP

phytanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyphytanoyl-CoA + succinate + CO2

Cofactor:

• iron, ascorbate

Structure

belongs to the phyH family

Compartment

peroxisome

Expression

• expressed in liver, kidney, & T-cells
• not expressed in spleen, brain, heart, lung & skeletal muscle

Pathology

• defects in PHYH are a cause of Refsum disease

Laboratory

• phytanoyl CoA hydroxlase in fibroblasts

More general terms

• oxidoreductase

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=5264
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5264
• OMIM: https://mirror.omim.org/entry/266500
• OMIM: https://mirror.omim.org/entry/602026
• UniProt: http://www.uniprot.org/uniprot/O14832.html