Names & Taxonomy

Uniprot ID:
P31327
Entry Name:
CPSM_HUMAN
Status:
reviewed
Protein Names:
Carbamoyl-phosphate synthase [ammonia], mitochondrial (EC 6.3.4.16) (Carbamoyl-phosphate synthetase I) (CPSase I)
Gene Names:
CPS1
Gene Names Primary:
CPS1
Organism:
Homo sapiens (Human)

Structure

Length:
1500
Sequence:
MTRILTAFKVVRTLKTGFGFTNVTAHQKWKFSRPGIRLLSVKAQTAHIVLEDGTKMKGYSFGHPSSVAGEVVFNTGLGGYPEAITDPAYKGQILTMANPIIGNGGAPDTTALDELGLSKYLESNGIKVSGLLVLDYSKDYNHWLATKSLGQWLQEEKVPAIYGVDTRMLTKIIRDKGTMLGKIEFEGQPVDFVDPNKQNLIAEVSTKDVKVYGKGNPTKVVAVDCGIKNNVIRLLVKRGAEVHLVPWNHDFTKMEYDGILIAGGPGNPALAEPLIQNVRKILESDRKEPLFGISTGNLITGLAAGAKTYKMSMANRGQNQPVLNITNKQAFITAQNHGYALDNTLPAGWKPLFVNVNDQTNEGIMHESKPFFAVQFHPEVTPGPIDTEYLFDSFFSLIKKGKATTITSVLPKPALVASRVEVSKVLILGSGGLSIGQAGEFDYSGSQAVKAMKEENVKTVLMNPNIASVQTNEVGLKQADTVYFLPITPQFVTEVIKAEQPDGLILGMGGQTALNCGVELFKRGVLKEYGVKVLGTSVESIMATEDRQLFSDKLNEINEKIAPSFAVESIEDALKAADTIGYPVMIRSAYALGGLGSGICPNRETLMDLSTKAFAMTNQILVEKSVTGWKEIEYEVVRDADDNCVTVCNMENVDAMGVHTGDSVVVAPAQTLSNAEFQMLRRTSINVVRHLGIVGECNIQFALHPTSMEYCIIEVNARLSRSSALASKATGYPLAFIAAKIALGIPLPEIKNVVSGKTSACFEPSLDYMVTKIPRWDLDRFHGTSSRIGSSMKSVGEVMAIGRTFEESFQKALRMCHPSIEGFTPRLPMNKEWPSNLDLRKELSEPSSTRIYAIAKAIDDNMSLDEIEKLTYIDKWFLYKMRDILNMEKTLKGLNSESMTEETLKRAKEIGFSDKQISKCLGLTEAQTRELRLKKNIHPWVKQIDTLAAEYPSVTNYLYVTYNGQEHDVNFDDHGMMVLGCGPYHIGSSVEFDWCAVSSIRTLRQLGKKTVVVNCNPETVSTDFDECDKLYFEELSLERILDIYHQEACGGCIISVGGQIPNNLAVPLYKNGVKIMGTSPLQIDRAEDRSIFSAVLDELKVAQAPWKAVNTLNEALEFAKSVDYPCLLRPSYVLSGSAMNVVFSEDEMKKFLEEATRVSQEHPVVLTKFVEGAREVEMDAVGKDGRVISHAISEHVEDAGVHSGDATLMLPTQTISQGAIEKVKDATRKIAKAFAISGPFNVQFLVKGNDVLVIECNLRASRSFPFVSKTLGVDFIDVATKVMIGENVDEKHLPTLDHPIIPADYVAIKAPMFSWPRLRDADPILRCEMASTGEVACFGEGIHTAFLKAMLSTGFKIPQKGILIGIQQSFRPRFLGVAEQLHNEGFKLFATEATSDWLNANNVPATPVAWPSQEGQNPSLSSIRKLIRDGSIDLVINLPNNNTKFVHDNYVIRRTAVDSGIPLLTNFQVTKLFAEAVQKSRKVDSKSLFHYRQYSAGKAA
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion

Function

Function:
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Catalytic Activity:
2 ATP + NH(3) + HCO(3)(-) = 2 ADP + phosphate + carbamoyl phosphate.
Kinetics:
BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=0.47 mM for ATP
Enzyme Regulation:
ENZYME REGULATION: Requires N-acetyl-L-glutamate (NAG) as an allosteric activator. Activated by glycerol in the absence of NAG, whereas in the presence of NAG it is inhibited by increasing concentrations of glycerol.
Cross Reference Drug Bank:
DB06775
Gene Ontology Go:
mitochondrial inner membrane
mitochondrial matrix
mitochondrial nucleoid
nucleolus
protein complex
aspartate carbamoyltransferase activity
ATP binding
calcium ion binding
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity
dihydroorotase activity
endopeptidase activity
glutamate binding
modified amino acid binding
phospholipid binding
'de novo' pyrimidine nucleobase biosynthetic process
anion homeostasis
arginine biosynthetic process
carbamoyl phosphate biosynthetic process
cellular nitrogen compound metabolic process
cellular response to cAMP
cellular response to fibroblast growth factor stimulus
cellular response to glucagon stimulus
cellular response to oleic acid
citrulline biosynthetic process
glutamine metabolic process
glycogen catabolic process
hepatocyte differentiation
homocysteine metabolic process
midgut development
nitric oxide metabolic process
polyamine metabolic process
positive regulation of vasodilation
response to amine
response to amino acid
response to dexamethasone
response to drug
response to food
response to growth hormone
response to lipopolysaccharide
response to starvation
response to toxic substance
response to zinc ion
small molecule metabolic process
triglyceride catabolic process
urea cycle
Gene Ontology Biological Process:
'de novo' pyrimidine nucleobase biosynthetic process
anion homeostasis
arginine biosynthetic process
carbamoyl phosphate biosynthetic process
cellular nitrogen compound metabolic process
cellular response to cAMP
cellular response to fibroblast growth factor stimulus
cellular response to glucagon stimulus
cellular response to oleic acid
citrulline biosynthetic process
glutamine metabolic process
glycogen catabolic process
hepatocyte differentiation
homocysteine metabolic process
midgut development
nitric oxide metabolic process
polyamine metabolic process
positive regulation of vasodilation
response to amine
response to amino acid
response to dexamethasone
response to drug
response to food
response to growth hormone
response to lipopolysaccharide
response to starvation
response to toxic substance
response to zinc ion
small molecule metabolic process
triglyceride catabolic process
urea cycle
Gene Ontology Molecular Function:
aspartate carbamoyltransferase activity
ATP binding
calcium ion binding
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity
dihydroorotase activity
endopeptidase activity
glutamate binding
modified amino acid binding
phospholipid binding
Gene Ontology Cellular Component:
mitochondrial inner membrane
mitochondrial matrix
mitochondrial nucleoid
nucleolus
protein complex
Keywords:
3D-structure
ATP-binding
Acetylation
Allosteric enzyme
Alternative splicing
Complete proteome
Disease mutation
Glycoprotein
Ligase
Mitochondrion
Nucleotide-binding
Nucleus
Phosphoprotein
Polymorphism
Reference proteome
Repeat
Transit peptide
Urea cycle
Interacts With:
P10398; P04049; P63104

Publication

PubMed ID:
1840546 9711878 12853138 12955727 12655559 14702039 15815621 15489334 17974005 19754428 21269460 22002106 24703693 24275569 11388595 11474210 11407344 15617192 15164414 16737834 17310273 20578160 20520828 21120950 21767969 22173106 23649895 24237036 24813853 26440671