ENTRY       EC 4.2.1.75
NAME        Uroporphyrinogen-III synthase
CLASS       Lyases
            Carbon-oxygen lyases
            Hydro-lyases
SYSNAME     Hydroxymethylbilane hydro-lyase(cyclizing)
REACTION    Hydroxymethylbilane = Uroporphyrinogen III + H2O
SUBSTRATE   Hydroxymethylbilane
PRODUCT     Uroporphyrinogen III
            H2O
COMMENT     In the presence of EC 4.3.1.8, forms uroporphyrinogen III from
            Porphobillinogen. (EC 4.3.1.8 Porphobillinogen deaminase)
PATHWAY     PATH: MAP00860  Porphyrin and chlorophyll metabolism
DISEASE     MIM: 263700  Uroporphyrinogen III synthase; Porphyria, congenital
                         erythropoietic (3)
GENES       MJA: MJ0994
            ECO: b3804(hemD)
            BSU: hemD
            SYN: sll0166(hemD)
            SCE: YOR278W(HEM4)
            HSA: UROS(Hs.75593)
DBLINKS     University of Geneva ENZYME DATA BANK: 4.2.1.75
            WIT (What Is There) Metabolic Reconstruction: 4.2.1.75
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