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Literature summary extracted from

  • Ahuja, V.; Powers-Lee, S.G.
    Human carbamoyl-phosphate synthetase: insight into N-acetylglutamate interaction and the functional effects of a common single nucleotide polymorphism (2008), J. Inherit. Metab. Dis., 31, 481-491.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
6.3.4.16 N-acetylglutamate the enzyme requires allosteric activation by N-acetylglutamate Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
6.3.4.16 gene CPSI, DNA and amino acid sequence determination and analysis, expression of the N-terminally His6-FLAG-tagged enzyme and mutant T1406D in Schizosaccharomyces pombe from pESP5, expression of mature protein of 1462 amino acid residues with the first 39 amino acids of the precursor protein replaced by a methionyl residue Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
6.3.4.16 additional information connstruction of a domain A deleted mutant hCPS_DELTAA and functional characterization, the mutant shows slightly inccreased catalytic efficiency in absence of N-acetylglutamate and a 4fold increased Km for N-acetylglutamate compared to the wild-type enzyme Homo sapiens
6.3.4.16 T1406D naturally occurring single nucleotide polymorphism, phenotype, overview Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
6.3.4.16 additional information
-
 additional information kinetics, the Km of the enzyme for ammonia is 100fold lower than that for CPSs that also use glutamine, EC 6.3.5.5, kinetics for bicarbonate-dependent ATPase and carbamoyl phosphate-dependent ATP synthesis reactions, overview Homo sapiens
6.3.4.16 0.29
-
 N-acetylglutamate pH 7.6, 37°C, wild-type enzyme Homo sapiens
6.3.4.16 0.35
-
 NH4+ pH 7.6, 37°C, wild-type enzyme Homo sapiens
6.3.4.16 0.44
-
 N-acetylglutamate pH 7.6, 37°C, mutant T1406D Homo sapiens
6.3.4.16 0.55
-
 ATP pH 7.6, 37°C, wild-type enzyme Homo sapiens
6.3.4.16 0.58
-
 NH4+ pH 7.6, 37°C, mutant T1406D Homo sapiens
6.3.4.16 0.68
-
 ATP pH 7.6, 37°C, mutant T1406D Homo sapiens
6.3.4.16 2.44
-
 HCO3- pH 7.6, 37°C, wild-type enzyme Homo sapiens
6.3.4.16 2.49
-
 HCO3- pH 7.6, 37°C, mutant T1406D Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
6.3.4.16 mitochondrial matrix the N-terminal targeting signal normally directs the nuclear-encoded protein to the mitochondrial matrix and is cleaved as the protein enters the matrix Homo sapiens 5759
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
6.3.4.16 Mg2+
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
6.3.4.16 ATP + NH4+ + HCO3- + H2O Homo sapiens the human enzyme removes excess, potentially neurotoxic ammonia via the urea cycle in a tightly regulated manner that avoids depletion of the ammonia pool, and shows inability to use glutamine as an alternative nitrogen donor ADP + phosphate + carbamoyl phosphate
-
 ?
6.3.4.16 additional information Homo sapiens severe hyperammonaemia and high mortality are observed in hCPS deficiency, a rare autosomal disease ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
6.3.4.16 Homo sapiens P31327 gene CPSI
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
6.3.4.16 liver
-
 Homo sapiens
-
6.3.4.16 small intestine
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
6.3.4.16 2 ATP + NH3 + CO2 + H2O
-
 Homo sapiens 2 ADP + phosphate + carbamoyl phosphate
-
 ?
6.3.4.16 ATP + NH4+ + HCO3- + H2O
-
 Homo sapiens ADP + phosphate + carbamoyl phosphate
-
 ?
6.3.4.16 ATP + NH4+ + HCO3- + H2O the human enzyme removes excess, potentially neurotoxic ammonia via the urea cycle in a tightly regulated manner that avoids depletion of the ammonia pool, and shows inability to use glutamine as an alternative nitrogen donor Homo sapiens ADP + phosphate + carbamoyl phosphate
-
 ?
6.3.4.16 additional information severe hyperammonaemia and high mortality are observed in hCPS deficiency, a rare autosomal disease Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
6.3.4.16 arginine-specific CPS
-
 Homo sapiens
6.3.4.16 carbamoyl-phosphate synthetase
-
 Homo sapiens
6.3.4.16 CPS
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
6.3.4.16 37
-
 assay at Homo sapiens

Turnover Number [1/s]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
6.3.4.16 5.54
-
 NH3 pH 7.6, 37°C, mutant T1406D Homo sapiens
6.3.4.16 5.73
-
 NH3 pH 7.6, 37°C, wild-type enzyme Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
6.3.4.16 7.6
-
 assay at Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
6.3.4.16 ATP
-
 Homo sapiens