2. Gene
  3. Cps1 - carbamoyl-phosphate synthetase 1 Gene

Cps1 - carbamoyl-phosphate synthetase 1 Gene

  • Gene
  • Protein
  • Agent
  • Ortholog
Mus musculus

Also known as CPS; D1Ucla3; 4732433M03Rik

Gene ID: 227231 | Gene type: protein coding

About Cps1

Summary

This gene encodes a protein localized to the inner mitochondrial matrix. The encoded protein plays a role in the detoxification of ammonia by catalyzing the first step in the urea cycle in which carbomyl-phosphate is synthesized from ammonia and bicarbonate. Carbamoyl-phosphate is subsequently converted to urea that is excreted by the kidneys. Deficiency of the encoded Enzyme leads to an accumulation of ammonia in the blood. High levels of ammonia are toxic to the central nervous system and result in neurological disorders. [provided by RefSeq, Oct 2013]

Cps1 Products(1)

mRNA Protein Name
NM_001080809.2 NP_001074278.1 carbamoyl-phosphate synthase [ammonia], mitochondrial precursor
Gene Ontology
  • Molecular Function
  • Cellular Component
Molecular Function GO Annotation Evidence Reference Source
enables carbamoyl-phosphate synthase (ammonia) activity IMP
IMP: Inferred from mutant phenotype
9862865 MGI
enables protein binding IPI
IPI: Inferred from physical interaction
19410549 MGI
Cellular Component GO Annotation Evidence Reference Source
located in mitochondrion IDA
IDA: Inferred from direct assay
6200105 MGI
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern
Protein Preferred Names Protein Names

carbamoyl-phosphate synthase [ammonia], mitochondrial

CPSase I

carbamoyl-phosphate synthetase I

carbamoylphosphate synthetase I

Cps1 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
Cps1 Q8C196 Sirt5 Mus musculus Q8K2C6
Anti Bait CoIP
19410549
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Agents

Approval
Bioactive Molecules (2)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-B0711 Carglumic Acid Activator 99.84% Yes
HY-B0711R Carglumic Acid (Standard) Activator / Unkown
Pre-clinical Phase
Bioactive Molecules (3)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-136128 H3B-120 Inhibitor 98.20% Unkown
HY-137199 Pro-Phe-Arg-AMC / Unkown
HY-RS03115 CPS1 Human Pre-designed siRNA Set A Pre-designed Sets / Unkown

Orthologs Information

Species Symbol Source ID
Homo sapiens Cps1 NCBI NCBI:1373