motifs/domains
| N-terminal signal peptide of 38 AAs, required for importing the protein into the mitochondria and is cleaved off by specific proteases after translocation |
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MGS a glutamine amido transferase-like |
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two ATP binding sites |
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a synthetase domain |
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structural domain I, II and III with unknown function, that are likely essentially involved in the preservation of the correct tertiary structure of the CPS1 monomer  |
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UFSD, which is in the middle of the 1462-AA multidomain CPS1 protein, plays a key integrating role for creating the CPS1 multidomain architecture leading us to propose here a denomination of "Integrating Domain" for this CPS1 region |
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a binding site for NBAS consists of a pocket that is located between the central beta-sheet and two alpha-helices (liver-specific, intramitochondrial, rate-limiting enzyme in the urea cycle) |
SUBCELLULAR LOCALIZATION
| intracellular
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| intracellular,cytoplasm,organelle,mitochondria,inner
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| intracellular,cytoplasm,organelle,mitochondria,matrix
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| intracellular,cytoplasm,organelle,membrane
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| intracellular,nucleus,nucleolus
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| basic FUNCTION
| playing an important role in removing excess ammonia from the cell |
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mitochondrial matrix enzyme that catalyzes the first step of the urea cycle, synthesis of carbamoylphosphate from bicarbonate, ATP, and ammonia |
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plays a paramount role in liver ureagenesis since it catalyzes the first and rate-limiting step of the urea cycle, the major pathway for nitrogen disposal ( |
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liver-specific, intramitochondrial, rate-limiting enzyme in the urea cycle |
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responsible for ammonia removal in the urea cycle, and nitration of CPS1 with attenuated function might be involved in some diseases and drug-induced toxicities associated with mitochondrial dysfunction |
