Lysyl hydroxylase

Lysyl hydroxylase (or procollagen-lysine 5-dioxygenase) is an oxygenase enzyme that catalyzes the hydroxylation of lysine to hydroxylysine. This reaction is necessary to the formation and stabilization of collagen. It takes place following protein synthesis (as a post-translational modification). The protein is a membrane-bound homodimeric enzyme that is localized to the cisternae (lumen) of the rough endoplasmic reticulum.

It requires iron and vitamin C as cofactors.

Pathology
A deficiency is associated with Ehlers-Danlos syndrome (type 6).