Protein: Carbamoyl-phosphate synthase [ammonia], mitochondrial (EC 6.3.4.16) (Carbamoyl-phosphate synthetase I) (CPSase I) (P07756)
Organism: Rattus norvegicus (Rat)
Function: Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Subcellular location: Mitochondrion. Nucleus, nucleolus {ECO:0000250|UniProtKB:P31327}. Cell membrane {ECO:0000250|UniProtKB:Q8C196}; Peripheral membrane protein {ECO:0000305}; Extracellular side {ECO:0000250|UniProtKB:Q8C196}. Note=Localizes to the cell surface of hepatocytes. {ECO:0000250|UniProtKB:Q8C196}.
Sequence: MTRILTACKVVKTLKSGFGLANVTSKRQWDFSRPGIRLLSVKAQTAHIVLEDGTKMKGYSFGHPSSVAGEVVFNTGLGGYSEALTDPAYKGQILTMANPIIGNGGAPDTTARDELGLNKYMESDGIKVAGLLVLNYSHDYNHWLATKSLGQWLQEEKVPAIYGVDTRMLTKIIRDKGTMLGKIEFEGQSVDFVDPNKQNLIAEVSTKDVKVFGKGNPTKVVAVDCGIKNNVIRLLVKRGAEVHLVPWNHDFTQMDYDGLLIAGGPGNPALAQPLIQNVKKILESDRKEPLFGISTGNIITGLAAGAKSYKMSMANRGQNQPVLNITNRQAFITAQNHGYALDNTLPAGWKPLFVNVNDQTNEGIMHESKPFFAVQFHPEVSPGPTDTEYLFDSFFSLIKKGKGTTITSVLPKPALVASRVEVSKVLILGSGGLSIGQAGEFDYSGSQAVKAMKEENVKTVLMNPNIASVQTNEVGLKQADAVYFLPITPQFVTEVIKAERPDGLILGMGGQTALNCGVELFKRGVLKEYGVKVLGTSVESIMATEDRQLFSDKLNEINEKIAPSFAVESMEDALKAADTIGYPVMIRSAYALGGLGSGICPNKETLMDLGTKAFAMTNQILVERSVTGWKEIEYEVVRDADDNCVTVCNMENVDAMGVHTGDSVVVAPAQTLSNAEFQMLRRTSINVVRHLGIVGECNIQFALHPTSMEYCIIEVNARLSRSSALASKATGYPLAFIAAKIALGIPLPEIKNVVSGKTSACFEPSLDYMVTKIPRWDLDRFHGTSSRIGSSMKSVGEVMAIGRTFEESFQKALRMCHPSVDGFTPRLPMNKEWPANLDLRKELSEPSSTRIYAIAKALENNMSLDEIVKLTSIDKWFLYKMRDILNMDKTLKGLNSESVTEETLRQAKEIGFSDKQISKCLGLTEAQTRELRLKKNIHPWVKQIDTLAAEYPSVTNYLYVTYNGQEHDIKFDEHGIMVLGCGPYHIGSSVEFDWCAVSSIRTLRQLGKKTVVVNCNPETVSTDFDECDKLYFEELSLERILDIYHQEACNGCIISVGGQIPNNLAVPLYKNGVKIMGTSPLQIDRAEDRSIFSAVLDELKVAQAPWKAVNTLNEALEFANSVGYPCLLRPSYVLSGSAMNVVFSEDEMKRFLEEATRVSQEHPVVLTKFIEGAREVEMDAVGKEGRVISHAISEHVEDAGVHSGDATLMLPTQTISQGAIEKVKDATRKIAKAFAISGPFNVQFLVKGNDVLVIECNLRASRSFPFVSKTLGVDFIDVATKVMIGESVDEKHLPTLEQPIIPSDYVAIKAPMFSWPRLRDADPILRCEMASTGEVACFGEGIHTAFLKAMLSTGFKIPQKGILIGIQQSFRPRFLGVAEQLHNEGFKLFATEATSDWLNANNVPATPVAWPSQEGQNPSLSSIRKLIRDGSIDLVINLPNNNTKFVHDNYVIRRTAVDSGIALLTNFQVTKLFAEAVQKARTVDSKSLFHYRQYSAGKAA
InterPro domains: - IPR029062: Class I glutamine amidotransferase-like (homologous_superfamily) [192-402]
- IPR016185: Pre-ATP-grasp domain superfamily (homologous_superfamily) [419-1089]
- IPR035686: Carbamoyl-phosphate synthase small subunit, GATase1 domain (domain) [220-395]
- IPR005480: Carbamoyl-phosphate synthetase, large subunit oligomerisation domain (domain) [839-962]
- IPR011761: ATP-grasp fold (domain) [551-1284]
- IPR011607: Methylglyoxal synthase-like domain (domain) [1355-1500]
- IPR058047: Carbamoyl phosphate synthase, preATP-grasp domain (domain) [423-1084]
- IPR005483: Carbamoyl phosphate synthase, CPSase domain (domain) [434-812]
- IPR005479: Carbamoyl phosphate synthase, ATP-binding domain (domain) [546-1290]
- IPR002474: Carbamoyl-phosphate synthase small subunit, N-terminal domain (domain) [44-184]
- IPR017926: Glutamine amidotransferase (domain) [222-395]
- IPR036897: Carbamoyl-phosphate synthetase, large subunit oligomerisation domain superfamily (homologous_superfamily) [810-969]
- IPR036914: Methylglyoxal synthase-like domain superfamily (homologous_superfamily) [1357-1495]
- IPR006275: Carbamoyl-phosphate synthase, large subunit (family) [419-1476]
- IPR036480: Carbamoyl-phosphate synthase small subunit, N-terminal domain superfamily (homologous_superfamily) [43-197]
- IPR013815: ATP-grasp fold, subdomain 1 (homologous_superfamily) [1101-1170]
- IPR006274: Carbamoyl-phosphate synthase, small subunit (family) [44-402]
Interaction partners: - Ornithine transcarbamylase, mitochondrial
- Argininosuccinate synthase
- Glutamate dehydrogenase 1, mitochondrial
- Glutamine synthetase
- N-acetylglutamate synthase, mitochondrial
- Argininosuccinate lyase
- Glutaminase liver isoform, mitochondrial
- Glutaminase kidney isoform, mitochondrial
- Multifunctional protein CAD
- Dihydroorotate dehydrogenase
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016874 (ligase activity), GO:0016740 (transferase activity), GO:0016787 (hydrolase activity), GO:0005515 (protein binding), GO:0016741 (transferase activity, transferring one-carbon groups), GO:0016810 (hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds), GO:0042802 (identical protein binding), GO:0016879 (ligase activity, forming carbon-nitrogen bonds), GO:0016884 (carbon-nitrogen ligase activity, with glutamine as amido-N-donor), GO:0016812 (hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in cyclic amides), GO:0016743 (carboxyl- or carbamoyltransferase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0051179 (localization), GO:0044419 (biological process involved in interspecies interaction between organisms), GO:0032501 (multicellular organismal process), GO:0065007 (biological regulation), GO:0050896 (response to stimulus), GO:0009987 (cellular process), GO:0009605 (response to external stimulus), GO:0009058 (biosynthetic process), GO:0042221 (response to chemical), GO:0006807 (nitrogen compound metabolic process), GO:0071704 (organic substance metabolic process), GO:0009056 (catabolic process), GO:0003008 (system process), GO:0065008 (regulation of biological quality), GO:0044237 (cellular metabolic process), GO:0044281 (small molecule metabolic process), GO:0009607 (response to biotic stimulus), GO:0051235 (maintenance of location), GO:0051716 (cellular response to stimulus), GO:0051707 (response to other organism), GO:0044238 (primary metabolic process), GO:1901576 (organic substance biosynthetic process), GO:1901564 (organonitrogen compound metabolic process), GO:0043207 (response to external biotic stimulus), GO:0044248 (cellular catabolic process), GO:0019637 (organophosphate metabolic process), GO:0044255 (cellular lipid metabolic process), GO:0044249 (cellular biosynthetic process), GO:1901700 (response to oxygen-containing compound), GO:0006520 (amino acid metabolic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0003013 (circulatory system process), GO:0010033 (response to organic substance), GO:0019627 (urea metabolic process), GO:0006793 (phosphorus metabolic process), GO:0010035 (response to inorganic substance), GO:0006629 (lipid metabolic process), GO:1901698 (response to nitrogen compound), GO:0070887 (cellular response to chemical stimulus), GO:2001057 (reactive nitrogen species metabolic process), GO:0006082 (organic acid metabolic process), GO:0009617 (response to bacterium), GO:0051238 (sequestering of metal ion), GO:0043603 (amide metabolic process), GO:1901575 (organic substance catabolic process), GO:0071941 (nitrogen cycle metabolic process), GO:0006790 (sulfur compound metabolic process), GO:0090066 (regulation of anatomical structure size), GO:0003018 (vascular process in circulatory system), GO:0032496 (response to lipopolysaccharide), GO:0000096 (sulfur amino acid metabolic process), GO:0008015 (blood circulation), GO:0043436 (oxoacid metabolic process), GO:1901699 (cellular response to nitrogen compound), GO:0002237 (response to molecule of bacterial origin), GO:0043604 (amide biosynthetic process), GO:0006638 (neutral lipid metabolic process), GO:0006796 (phosphate-containing compound metabolic process), GO:0044242 (cellular lipid catabolic process), GO:0016042 (lipid catabolic process), GO:1901605 (alpha-amino acid metabolic process), GO:0035150 (regulation of tube size), GO:1901566 (organonitrogen compound biosynthetic process), GO:0000050 (urea cycle), GO:0046486 (glycerolipid metabolic process), GO:0033993 (response to lipid), GO:0090407 (organophosphate biosynthetic process), GO:0044271 (cellular nitrogen compound biosynthetic process), GO:0060359 (response to ammonium ion), GO:0046209 (nitric oxide metabolic process), GO:0050667 (homocysteine metabolic process), GO:0006639 (acylglycerol metabolic process), GO:0046503 (glycerolipid catabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0097746 (blood vessel diameter maintenance), GO:0035296 (regulation of tube diameter), GO:0046461 (neutral lipid catabolic process), GO:0042311 (vasodilation), GO:0006641 (triglyceride metabolic process), GO:0046464 (acylglycerol catabolic process), GO:0019433 (triglyceride catabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0032991 (protein-containing complex), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0150034 (distal axon), GO:0042995 (cell projection), GO:0016020 (membrane), GO:0044297 (cell body), GO:0005829 (cytosol), GO:0098793 (presynapse), GO:0009986 (cell surface), GO:0071944 (cell periphery), GO:0030054 (cell junction), GO:0044306 (neuron projection terminus), GO:0031975 (envelope), GO:0005615 (extracellular space), GO:0036477 (somatodendritic compartment), GO:0005737 (cytoplasm), GO:0005576 (extracellular region), GO:0031090 (organelle membrane), GO:0031967 (organelle envelope), GO:0120025 (plasma membrane bounded cell projection), GO:0043679 (axon terminus), GO:0043229 (intracellular organelle), GO:0045202 (synapse), GO:0043025 (neuronal cell body), GO:0005739 (mitochondrion), GO:0097447 (dendritic tree), GO:0043227 (membrane-bounded organelle), GO:0005886 (plasma membrane), GO:0005740 (mitochondrial envelope), GO:0043231 (intracellular membrane-bounded organelle), GO:0019866 (organelle inner membrane), GO:0031966 (mitochondrial membrane), GO:0005743 (mitochondrial inner membrane), GO:0030424 (axon)