InterPro domain: IPR008775

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 [ 1 ].

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) [ 2 ].

1. Human phytanoyl-CoA hydroxylase: resolution of the gene structure and the molecular basis of Refsum's disease. Hum. Mol. Genet. 9, 1195-200
2. PhnY and PhnZ comprise a new oxidative pathway for enzymatic cleavage of a carbon-phosphorus bond. J. Am. Chem. Soc. 134, 8364-7