Anti-Phytanoyl-CoA dioxygenase, peroxisomal precursor

PHYH (Phytanoyl-CoA 2-hydroxylase) is mainly involved in the catalytic hydroxylation of phytanoyl-CoA. It facilitates the initial α-oxidation step in phytenic acid degradation in peroxisomes. Deficiency of PHYH causes an inherited disorder of lipid metabolism, Refsum disease, characterized by the accumulation of phytanic acid.