ENTRY       EC 1.14.11.4
NAME        Procollagen-lysine 5-dioxygenase
            Lysine hydroxylase
            Lysine,2-oxoglutarate 5-dioxygenase
CLASS       Oxidoreductases
            Acting on paired donors with incorporation of molecular oxygen
            With 2-oxoglutarate as one donor, and incorporation of one atom
            each of oxygen into both donors
SYSNAME     Procollagen-L-lysine,2-oxoglutarate:oxygen oxidoreductase
            (5-hydroxylating)
REACTION    Procollagen L-lysine + 2-Oxoglutarate + O2 =
            Procollagen 5-hydroxy-L-lysine + Succinate + CO2
SUBSTRATE   Procollagen L-lysine
            2-Oxoglutarate
            O2
            L-Lysine
PRODUCT     Procollagen 5-hydroxy-L-lysine
            Succinate
            CO2
            5-Hydroxy-L-lysine
COFACTOR    Iron
            Ascorbate
COMMENT     Requires Fe2+ and ascorbate.
PATHWAY     PATH: MAP00310  Lysine degradation
DISEASE     MIM: 153454  Procollagen-lysine, 2-oxoglutarate 5-dioxygenase
                         (lysine hydroxylase); Ehlers-Danlos syndrome, type VI,
                         225400 (3)
GENES       HSA: PLOD(LLH)
DBLINKS     University of Geneva ENZYME DATA BANK: 1.14.11.4
            WIT (What Is There) Metabolic Reconstruction: 1.14.11.4
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