ENTRY       EC 1.14.11.2
NAME        Procollagen-proline dioxygenase
            Protocollagen hydroxylase
            Proline hydroxylase
            Proline,2-oxoglutarate 4-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-proline,2-oxoglutarate:oxygen oxidoreductase
            (4-hydroxylating)
REACTION    Procollagen L-proline + 2-Oxoglutarate + O2 =
            Procollagen trans-4-hydroxy-L-proline + Succinate + CO2
SUBSTRATE   Procollagen L-proline
            2-Oxoglutarate
            O2
            L-Proline
PRODUCT     Procollagen trans-4-hydroxy-L-proline
            Succinate
            CO2
            trans-4-Hydroxy-L-proline
COFACTOR    Iron
            Ascorbate
COMMENT     Requires Fe2+ and ascorbate.
PATHWAY     PATH: MAP00330  Arginine and proline metabolism
DISEASE     MIM: 176710  Procollagen-proline, 2-oxoglutarate 4-dioxygenase
                         (proline
MOTIF       PS: PS00014  [KRHQSA]-[DENQ]-E-L>
            PS: PS00194  [LIVMF]-[LIVMSTA]-x-[LIVMFYC]-[FYWSTHE]-x(2)-[FYWGTN]-
                         C-[GATPLVE]-[PHYSTA]-C-x(6)-[LIVMFYWT]
GENES       HSA: P4HA(Hs.89513) P4HB(PO4DB)
DBLINKS     University of Geneva ENZYME DATA BANK: 1.14.11.2
            WIT (What Is There) Metabolic Reconstruction: 1.14.11.2
///