6wfv

Structural highlights

Disease

PLOD3_HUMAN Connective tissue disorder due to lysyl hydroxylase-3 deficiency. The disease is caused by mutations affecting the gene represented in this entry.

Function

PLOD3_HUMAN Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.[UniProtKB:P24802]