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