Family

Phytanoyl-CoA dioxygenase (IPR008775)

Short name: Phytyl_CoA_dOase

Family relationships

Description

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].

Contributing signatures

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