PHYH : phytanoyl-CoA 2-hydroxylase
Description
The PHYH (phytanoyl-CoA 2-hydroxylase) is a protein-coding gene located on chromosome 10.
The PHYH gene provides instructions for creating an enzyme called phytanoyl-CoA hydroxylase. This enzyme is crucial for the proper functioning of peroxisomes, which are sac-like cell compartments containing enzymes necessary to break down various substances, including fatty acids and toxic compounds. Phytanic acid, a dietary fatty acid primarily found in beef and dairy products, is broken down in peroxisomes. Phytanoyl-CoA hydroxylase initiates this breakdown process through a step called alpha-oxidation. Subsequent steps involve other peroxisomal and cellular enzymes that further process phytanic acid into smaller molecules for energy utilization. Researchers believe that phytanoyl-CoA hydroxylase may have additional roles beyond phytanic acid breakdown, possibly influencing the number and activity of peroxisomes within cells.
PHYH catalyzes the 2-hydroxylation of a variety of substrates, including racemic phytanoyl-CoA, isomers of 3-methylhexadecanoyl-CoA, and other mono-branched 3-methylacyl-CoA esters (with at least 7 carbon atoms), as well as straight-chain acyl-CoA esters (with more than 4 carbon atoms). It does not, however, hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs. These activities have been confirmed by multiple studies (PubMed:9326939, PubMed:10744784, PubMed:12031666, PubMed:12923223).
PHYH is also known as LN1, LNAP1, PAHX, PHYH1, RD.
