InterPro domain: IPR039575

This entry represents prolyl 3-hydroxylase, including prolyl 3-hydroxylase catalyses 1-3 (P3H1-3) from humans. P3H1, also known as leprecan-1, is responsible for converting proline to 3-hydroxyproline (3Hyp) in type I collagen. P3H2 is responsible for 3-hydroxylating multiple proline sites in collagen types I, II, IV, and V [ 1 ]. However, P3H3 is not required for normal prolyl 3-hydroxylation on collagen chains [ 2 ].

1. Characterization of recombinant human prolyl 3-hydroxylase isoenzyme 2, an enzyme modifying the basement membrane collagen IV. J. Biol. Chem. 283, 19432-9
2. P3h3-null and Sc65-null Mice Phenocopy the Collagen Lysine Under-hydroxylation and Cross-linking Abnormality of Ehlers-Danlos Syndrome Type VIA. J. Biol. Chem. 292, 3877-3887