ENTRY       EC 4.2.1.96
NAME        Tetrahydrobiopterin dehydratase
            Pterin-4-alpha-carbinolamide dehydratase
            4-alpha-Hydroxy-tetrahydropterin dehydratase
CLASS       Lyases
            Carbon-oxygen lyases
            Hydro-lyases
REACTION    2-Amino-4-oxo-4-alpha-hydroxy-6-(erythro-1',2',3'-Tri-
            $hydroxypropyl)-5,6,7,8-tetrahydroxypterin = 2-Amino-4-oxo-
            $6-(1',2',3'-trihydroxypropyl)-diquinoid-7,8-dihydroxypterin
            + H2O
SUBSTRATE   2-Amino-4-oxo-4-alpha-hydroxy-6-(erythro-1',2',3'-Tri-
            $hydroxypropyl)-5,6,7,8-tetrahydroxypterin
PRODUCT     2-Amino-4-oxo-6-(1',2',3'-trihydroxypropyl)-diquinoid-
            $7,8-dihydroxypterin
            H2O
COMMENT     Catalyses the dehydratation of 4-alpha-Hydroxy-tetrahydro-
            pterines.
DISEASE     MIM: 126090  Dimerization cofactor of hepatic nuclear factor
                         1-alpha (TCF1); Hyperphenylalaninemia due to
                         pterin-4a-carbinolamine dehydratase deficiency, 264070
                         (3)
GENES       SYN: ssl2296(dcoH)
STRUCTURES  PDB: 1DCO  1DCP  
DBLINKS     University of Geneva ENZYME DATA BANK: 4.2.1.96
            WIT (What Is There) Metabolic Reconstruction: 4.2.1.96
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