PHYHD1 (phytanoyl-CoA dioxygenase domain containing 1) is a 291 amino acid protein belonging to the PHYH family and the PHYHD1 subfamily. Encoded by a gene that maps to human chromosome 9q34.11, PHYHD1 exists as three alternatively spliced isoforms and likely functions as an alpha-ketoglutatare-dependent dioxygenase. PHYHD1 participates in metal ion binding and oxidoreductase activity, thereby acting on single donors with incorporation of two atoms of oxygen. Related to PHYH (phytanoyl-CoA 2-hydroxylase), an Fe(II) and 2-oxoglutarate (2OG)-dependent oxygenase that catalyzes the initial alpha-oxidation step in the degradation of phytenic acid in peroxisomes, PHYHD1 similarly exhibits additional homologues in a wide range of metazoans and bacteria. PHYHD1 also may play a role in DNA methylation in early postnatal liver development and mammalian differentiation.
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