MGP Database

MGP000671

Ontology/Pathway Information

Entrez Gene ID	1373
Gene Name	carbamoyl-phosphate synthase 1, mitochondrial
Gene Symbol	CPS1
Species	Homo sapiens

Gene Ontology (GO Annotations)

GO ID	Source	Type	Description
GO:0005743	IEA:Ensembl	C	mitochondrial inner membrane
GO:0005759	TAS:Reactome	C	mitochondrial matrix
GO:0042645	IDA:BHF-UCL	C	mitochondrial nucleoid
GO:0005730	IEA:UniProtKB-SubCell	C	nucleolus
GO:0043234	IEA:Ensembl	C	protein complex
GO:0005524	IEA:UniProtKB-KW	F	ATP binding
GO:0005509	IEA:Ensembl	F	calcium ion binding
GO:0004087	IMP:BHF-UCL	F	carbamoyl-phosphate synthase (ammonia) activity
GO:0004088	IBA:GO_Central	F	carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity
GO:0004175	IEA:Ensembl	F	endopeptidase activity
GO:0016595	IEA:Ensembl	F	glutamate binding
GO:0072341	IDA:UniProtKB	F	modified amino acid binding
GO:0005543	IEA:Ensembl	F	phospholipid binding
GO:0055081	IEA:Ensembl	P	anion homeostasis
GO:0006526	IBA:GO_Central	P	arginine biosynthetic process
GO:0070409	IMP:UniProtKB	P	carbamoyl phosphate biosynthetic process
GO:0034641	TAS:Reactome	P	cellular nitrogen compound metabolic process
GO:0071320	IEA:Ensembl	P	cellular response to cAMP
GO:0044344	IEA:Ensembl	P	cellular response to fibroblast growth factor stimulus
GO:0071377	IEA:Ensembl	P	cellular response to glucagon stimulus
GO:0071400	IEA:Ensembl	P	cellular response to oleic acid
GO:0019240	NAS:BHF-UCL	P	citrulline biosynthetic process
GO:0006543	IEA:InterPro	P	glutamine catabolic process
GO:0005980	IMP:BHF-UCL	P	glycogen catabolic process
GO:0070365	IEA:Ensembl	P	hepatocyte differentiation
GO:0050667	IDA:UniProtKB	P	homocysteine metabolic process
GO:0007494	IEA:Ensembl	P	midgut development
GO:0046209	IMP:BHF-UCL	P	nitric oxide metabolic process
GO:0045909	IMP:BHF-UCL	P	positive regulation of vasodilation
GO:0014075	IEA:Ensembl	P	response to amine
GO:0043200	IEA:Ensembl	P	response to amino acid
GO:0071548	IEA:Ensembl	P	response to dexamethasone
GO:0042493	IEA:Ensembl	P	response to drug
GO:0032094	IEA:Ensembl	P	response to food
GO:0060416	IEA:Ensembl	P	response to growth hormone
GO:0032496	IDA:UniProtKB	P	response to lipopolysaccharide
GO:0042594	IEA:Ensembl	P	response to starvation
GO:0009636	IEA:Ensembl	P	response to toxic substance
GO:0010043	IEA:Ensembl	P	response to zinc ion
GO:0044281	TAS:Reactome	P	small molecule metabolic process
GO:0019433	IMP:BHF-UCL	P	triglyceride catabolic process
GO:0000050	NAS:BHF-UCL	P	urea cycle

REACTOME Pathway Links

REACTOME Pathway ID	Description
REACT_111217	Metabolism
REACT_13	Metabolism of amino acids and derivatives
REACT_847	Urea cycle

SMP Pathway Links

SMP ID	Description
SMP00136	2-Hydroxyglutric Aciduria (D And L Form)
SMP00243	4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
SMP00009	Ammonia Recycling
SMP00020	Arginine and Proline Metabolism
SMP00362	Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
SMP00357	Argininemia
SMP00003	Argininosuccinic Aciduria
SMP00002	Carbamoyl Phosphate Synthetase Deficiency
SMP00001	Citrullinemia Type I
SMP00504	Creatine deficiency, guanidinoacetate methyltransferase deficiency
SMP00072	Glutamate Metabolism
SMP00188	Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
SMP00385	Homocarnosinosis
SMP00339	Hyperinsulinism-Hyperammonemia Syndrome
SMP00506	Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]
SMP00505	Hyperornithinemia with gyrate atrophy (HOGA)
SMP00361	Hyperprolinemia Type I
SMP00360	Hyperprolinemia Type II
SMP00507	L-arginine:glycine amidinotransferase deficiency
SMP00363	Ornithine Aminotransferase Deficiency (OAT Deficiency)
SMP00205	Ornithine Transcarbamylase Deficiency (OTC Deficiency)
SMP00207	Prolidase Deficiency (PD)
SMP00208	Prolinemia Type II
SMP00567	Succinic semialdehyde dehydrogenase deficiency
SMP00059	Urea Cycle