carbamoyl-phosphate synthase 1

Summary
Gene Symbol
  • CPS1
Organism
Homo sapiens (human)
NCBI Gene
1373
KEGG Gene ID
PubChem
1373
Alliance of Genome Resources
Annotation
Keyword
  • 3D-structure
  • ATP-binding
  • Acetylation
  • Allosteric enzyme
  • Alternative splicing
  • Cell membrane
  • Disease variant
  • Glycoprotein
  • Ligase
  • Mitochondrion
  • Nucleus
  • Phosphoprotein
  • Proteomics identification
  • Reference proteome
  • Repeat
  • Transit peptide
  • Urea cycle
Proteins
Displaying all 2 entries
UniProt Protein Name
P31327
  • Carbamoyl-phosphate synthetase I
Q6PEK7
Gene Ontology (GO)
Displaying entries 1 - 5 of 39 in total
GO Term Evidence Code PMID
urea cycle
urea cycle
urea cycle
liver development
'de novo' pyrimidine nucleobase biosynthetic process
GO Hierarchy
GO Hierarchy
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
Disease
Disease Ontology
Displaying all 3 entries
DO ID Disease Name Source
DOID:9280 carbamoyl phosphate synthetase I deficiency disease
DOID:13042 persistent fetal circulation syndrome
DOID:178 vascular disease
Ortholog
Displaying all 2 entries
Species Gene ID Alliance of Genome Resources
227231 MGI:891996
497840 RGD:2395

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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