| lysyl hydroxylase |
an enzyme of the oxidoreductase class that catalyzes the hydroxylation of specific lysine residues in nascent procollagen chains; the hydroxylysine residues act as sites of attachment for disaccharide prosthetic groups and are involved in the formation of strong interchain crosslinks in collagen. The enzyme requires Fe2+, ascorbate, and α-ketoglutarate for activity. Deficiency of enzyme activity, an autosomal recessive trait, results in Ehlers-Danlos syndrome, type VI. In EC nomenclature, called procollagen-lysine 5-dioxygenase.
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