P3H3

Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils. Required for normal hydroxylation of lysine residues in type I collagen chains in skin, bone, tendon, aorta and cornea. Required for normal skin stability via its role in hydroxylation of lysine residues in collagen alpha chains and in collagen fibril assembly. Apparently not required for normal prolyl 3-hydroxylation on collagen chains, either because a proline substrate for P3H3 remains to be identified or either is lost enzyme activity over the course of evolution. Also requires for lysyl hydroxylation of type V collagen in the skin, either by supporting hydroxylation of type V collagen by PLOD2/LH2 or by having itself a lysyl hydroxylase activity..

Function from UniProtKB (Q8IVL6), read 2026-09-23. CC BY 4.0.