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carbamoyl-phosphate synthetase 1

Summary
Gene Symbol
  • Cps1
Organism
Mus musculus (house mouse)
NCBI Gene
227231
KEGG Gene ID
PubChem
227231
Alliance of Genome Resources
Annotation
Keyword
  • ATP-binding
  • Acetylation
  • Allosteric enzyme
  • Cell membrane
  • Direct protein sequencing
  • Glycoprotein
  • Ligase
  • Mitochondrion
  • Nucleus
  • Phosphoprotein
  • Reference proteome
  • Repeat
  • Transit peptide
  • Urea cycle
Proteins
Displaying 1 entry
UniProt Protein Name
Q8C196
  • Carbamoyl-phosphate synthetase I
Annotation information from UniProt.
Gene Ontology (GO)
Displaying entries 1 - 5 of 44 in total
GO Term Evidence Code PMID
urea cycle
urea cycle
urea cycle
liver development
'de novo' pyrimidine nucleobase biosynthetic process
GO Hierarchy
Displaying entries 1 - 5 of 25 in total
GO Term Evidence Code PMID
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity
GO Hierarchy
Gene Ontology information from NCBI Gene and UniProt.
OrthoDB (Group)
Group level
Eukaryota
Group Name
Carbamoyl-phosphate
Functional Category
  • E: Amino acid transport and metabolism
  • J: Translation, ribosomal structure and biogenesis
  • T: Signal transduction mechanisms
Displaying all 5 entries
Gene Ontology
ATP binding
carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity
glutamine metabolic process
ligase activity
nucleotide binding
Disease
Disease Ontology
Displaying 1 entry
DO ID Disease Name Source
DOID:9280 carbamoyl phosphate synthetase I deficiency disease
Ortholog
Displaying all 2 entries
Species Gene ID Alliance of Genome Resources
497840 RGD:2395
1373 HGNC:2323
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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: April 6, 2026