Synonyms: 2-oxoglutarate 5-dioxygenase 3,bone protein I,BP-I,LH3,Lysine hydroxylase 3,Lysyl hydroxylase 3,Plod3,PLOD3,Procollagen lysine,2 oxoglutarate 5 dioxygenase 3 precursor,Procollagen-lysine
Background: The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VIB have deficiencies in lysyl hydroxylase activity.