Phytanoyl-CoA dioxygenase (IPR008775)

Short name: Phytyl_CoA_dOase

Overlapping homologous superfamilies


Family relationships


This family is made up of several eukaryotic phytanoyl-CoA dioxygenase (PhyH) proteins as well as a number of bacterial deoxygenases. PhyH is a peroxisomal enzyme catalysing the first step of phytanic acid alpha-oxidation. PhyH deficiency causes Refsum's disease (RD) which is an inherited neurological syndrome biochemically characterised by the accumulation of phytanic acid in plasma and tissues [PMID: 10767344].

The bacterial deoxygenase 2-aminoethylphosphonate dioxygenase (PhnY) hydroxyles 2-aminoethylphosphonic acid to form (2-amino-1-hydroxyethyl)phosphonic acid, which is then oxidatively converted to inorganic phosphate and glycine by 2-amino-1-hydroxyethylphosphonate dioxygenase (PhnZ) [PMID: 22564006].

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.