The CPS-I reaction is a preparatory step, the condensation and activation of HCO3– and NH4+. CPS-I is localised in the mitochondria and catalyses a two-step reaction (Figure 1).

Production of Carbamoyl - Phosphate (CP)

  1. HCO3- is activated by using ATP
  2. followed by a Pi-NH4+ exchange

In the second step

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