1. CPSI is in mitochondria, and CPSII is in cytosolic. 2. Carbamoyl phosphate in enzyme. 3. So the carbamoyl phosphate formed in mitochondria can be used only for argenine synthesis, and that formed in cytosol is used only for pyrimidine synthesis.
Despite the fact that both CPS activities are easily found in cells like periportal hepatocytes, the CPS-2 activity associated with CAD is found in the cytoplasm, whilst the CPS-1 activity is unique to mitochondria, where it largely participates in the urea cycle. The initial phase of the urea cycle, which is where more nitrogen molecules are added to the cycle to be digested, is controlled by the carbamoyl phosphate synthetase I enzyme.
The hereditary condition known as carbamoyl phosphate synthetase I deficiency is one of several urea cycle abnormalities. Using a catalytic process that passes via three reactive intermediaries—carboxy phosphate, ammonia, and carbamate—CPS produces carbamoyl phosphate from two molecules of MgATP, one molecule of bicarbonate, and one molecule of glutamine.
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