ENTRY       EC 6.3.4.16
NAME        Carbamoyl-phosphate synthase (ammonia)
            Carbon-dioxide--ammonia ligase
CLASS       Ligases
            Forming carbon-nitrogen bonds
            Other carbon--nitrogen ligases
SYSNAME     Carbon-dioxide:ammonia ligase (ADP-forming,
            carbamate-phosphorylating)
REACTION    2 ATP + NH3 + CO2 + H2O = 2 ADP + Orthophosphate +
            Carbamoyl phosphate
SUBSTRATE   ATP
            NH3
            CO2
            H2O
PRODUCT     ADP
            Orthophosphate
            Carbamoyl phosphate
EFFECTOR    N-Acetyl-L-glutamate
COMMENT     Formerly EC 2.7.2.5.  Requires N-acetylglutamate as allosgteric
            activator.
PATHWAY     PATH: MAP00220  Urea cycle and metabolism of amino groups
            PATH: MAP00251  Glutamate metabolism
            PATH: MAP00330  Arginine and proline metabolism
            PATH: MAP00910  Nitrogen metabolism
DISEASE     MIM: 237300  Carbamoyl-phosphate synthetase 1, mitochondrial;
                         Carbamoylphosphate synthetase I deficiency (3)
MOTIF       PS: PS00866  [FYV]-P-[LIVMC]-[LIVMA]-[LIVM]-[KR]-[PSA]-[STA]-x(3)-
                         [SG]-G-x-[AG]
            PS: PS00867  [LIVMF]-[LIMN]-E-[LIVMCA]-N-[PATLIVM]-[KR]-[LIVMSTAC]
GENES       HSA: CPS1(Hs.50966)
DBLINKS     University of Geneva ENZYME DATA BANK: 6.3.4.16
            WIT (What Is There) Metabolic Reconstruction: 6.3.4.16
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