Carbamoyl-phosphate synthase [ammonia], mitochondrial

Details

Name
Carbamoyl-phosphate synthase [ammonia], mitochondrial
Synonyms
  • 6.3.4.16
  • Carbamoyl-phosphate synthetase I
  • CPSase I
Gene Name
CPS1
Organism
Humans
Amino acid sequence
>lcl|BSEQ0009589|Carbamoyl-phosphate synthase [ammonia], mitochondrial
MTRILTAFKVVRTLKTGFGFTNVTAHQKWKFSRPGIRLLSVKAQTAHIVLEDGTKMKGYS
FGHPSSVAGEVVFNTGLGGYPEAITDPAYKGQILTMANPIIGNGGAPDTTALDELGLSKY
LESNGIKVSGLLVLDYSKDYNHWLATKSLGQWLQEEKVPAIYGVDTRMLTKIIRDKGTML
GKIEFEGQPVDFVDPNKQNLIAEVSTKDVKVYGKGNPTKVVAVDCGIKNNVIRLLVKRGA
EVHLVPWNHDFTKMEYDGILIAGGPGNPALAEPLIQNVRKILESDRKEPLFGISTGNLIT
GLAAGAKTYKMSMANRGQNQPVLNITNKQAFITAQNHGYALDNTLPAGWKPLFVNVNDQT
NEGIMHESKPFFAVQFHPEVTPGPIDTEYLFDSFFSLIKKGKATTITSVLPKPALVASRV
EVSKVLILGSGGLSIGQAGEFDYSGSQAVKAMKEENVKTVLMNPNIASVQTNEVGLKQAD
TVYFLPITPQFVTEVIKAEQPDGLILGMGGQTALNCGVELFKRGVLKEYGVKVLGTSVES
IMATEDRQLFSDKLNEINEKIAPSFAVESIEDALKAADTIGYPVMIRSAYALGGLGSGIC
PNRETLMDLSTKAFAMTNQILVEKSVTGWKEIEYEVVRDADDNCVTVCNMENVDAMGVHT
GDSVVVAPAQTLSNAEFQMLRRTSINVVRHLGIVGECNIQFALHPTSMEYCIIEVNARLS
RSSALASKATGYPLAFIAAKIALGIPLPEIKNVVSGKTSACFEPSLDYMVTKIPRWDLDR
FHGTSSRIGSSMKSVGEVMAIGRTFEESFQKALRMCHPSIEGFTPRLPMNKEWPSNLDLR
KELSEPSSTRIYAIAKAIDDNMSLDEIEKLTYIDKWFLYKMRDILNMEKTLKGLNSESMT
EETLKRAKEIGFSDKQISKCLGLTEAQTRELRLKKNIHPWVKQIDTLAAEYPSVTNYLYV
TYNGQEHDVNFDDHGMMVLGCGPYHIGSSVEFDWCAVSSIRTLRQLGKKTVVVNCNPETV
STDFDECDKLYFEELSLERILDIYHQEACGGCIISVGGQIPNNLAVPLYKNGVKIMGTSP
LQIDRAEDRSIFSAVLDELKVAQAPWKAVNTLNEALEFAKSVDYPCLLRPSYVLSGSAMN
VVFSEDEMKKFLEEATRVSQEHPVVLTKFVEGAREVEMDAVGKDGRVISHAISEHVEDAG
VHSGDATLMLPTQTISQGAIEKVKDATRKIAKAFAISGPFNVQFLVKGNDVLVIECNLRA
SRSFPFVSKTLGVDFIDVATKVMIGENVDEKHLPTLDHPIIPADYVAIKAPMFSWPRLRD
ADPILRCEMASTGEVACFGEGIHTAFLKAMLSTGFKIPQKGILIGIQQSFRPRFLGVAEQ
LHNEGFKLFATEATSDWLNANNVPATPVAWPSQEGQNPSLSSIRKLIRDGSIDLVINLPN
NNTKFVHDNYVIRRTAVDSGIPLLTNFQVTKLFAEAVQKSRKVDSKSLFHYRQYSAGKAA
Number of residues
1500
Molecular Weight
164938.145
Theoretical pI
Not Available
GO Classification
Functions
ATP binding / calcium ion binding / carbamoyl-phosphate synthase (ammonia) activity / carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity / endopeptidase activity / glutamate binding / modified amino acid binding / phospholipid binding
Processes
'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
Components
cytoplasm / mitochondrial inner membrane / mitochondrial matrix / mitochondrial nucleoid / nucleolus / protein complex
General Function
Phospholipid binding
Specific Function
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Mitochondrion
Gene sequence
>lcl|BSEQ0013253|Carbamoyl-phosphate synthase [ammonia], mitochondrial (CPS1)
ATGCCACAAATCATCAAAATGACGAGGATTTTGACAGCTTTCAAAGTGGTGAGGACACTG
AAGACTGGTTTTGGCTTTACCAATGTGACTGCACACCAAAAATGGAAATTTTCAAGACCT
GGCATCAGGCTCCTTTCTGTCAAGGCACAGACAGCACACATTGTCCTGGAAGATGGAACT
AAGATGAAAGGTTACTCCTTTGGCCATCCATCCTCTGTTGCTGGTGAAGTGGTTTTTAAT
ACTGGCCTGGGAGGGTACCCAGAAGCTATTACTGACCCTGCCTACAAAGGACAGATTCTC
ACAATGGCCAACCCTATTATTGGGAATGGTGGAGCTCCTGATACTACTGCTCTGGATGAA
CTGGGACTTAGCAAATATTTGGAGTCTAATGGAATCAAGGTTTCAGGTTTGCTGGTGCTG
GATTATAGTAAAGACTACAACCACTGGCTGGCTACCAAGAGTTTAGGGCAATGGCTACAG
GAAGAAAAGGTTCCTGCAATTTATGGAGTGGACACAAGAATGCTGACTAAAATAATTCGG
GATAAGGGTACCATGCTTGGGAAGATTGAATTTGAAGGTCAGCCTGTGGATTTTGTGGAT
CCAAATAAACAGAATTTGATTGCTGAGGTTTCAACCAAGGATGTCAAAGTGTACGGCAAA
GGAAACCCCACAAAAGTGGTAGCTGTAGACTGTGGGATTAAAAACAATGTAATCCGCCTG
CTAGTAAAGCGAGGAGCTGAAGTGCACTTAGTTCCCTGGAACCATGATTTCACCAAGATG
GAGTATGATGGGATTTTGATCGCGGGAGGACCGGGGAACCCAGCTCTTGCAGAACCACTA
ATTCAGAATGTCAGAAAGATTTTGGAGAGTGATCGCAAGGAGCCATTGTTTGGAATCAGT
ACAGGAAACTTAATAACAGGATTGGCTGCTGGTGCCAAAACCTACAAGATGTCCATGGCC
AACAGAGGGCAGAATCAGCCTGTTTTGAATATCACAAACAAACAGGCTTTCATTACTGCT
CAGAATCATGGCTATGCCTTGGACAACACCCTCCCTGCTGGCTGGAAACCACTTTTTGTG
AATGTCAACGATCAAACAAATGAGGGGATTATGCATGAGAGCAAACCCTTCTTCGCTGTG
CAGTTCCACCCAGAGGTCACCCCGGGGCCAATAGACACTGAGTACCTGTTTGATTCCTTT
TTCTCACTGATAAAGAAAGGAAAAGCTACCACCATTACATCAGTCTTACCGAAGCCAGCA
CTAGTTGCATCTCGGGTTGAGGTTTCCAAAGTCCTTATTCTAGGATCAGGAGGTCTGTCC
ATTGGTCAGGCTGGAGAATTTGATTACTCAGGATCTCAAGCTGTAAAAGCCATGAAGGAA
GAAAATGTCAAAACTGTTCTGATGAACCCAAACATTGCATCAGTCCAGACCAATGAGGTG
GGCTTAAAGCAAGCGGATACTGTCTACTTTCTTCCCATCACCCCTCAGTTTGTCACAGAG
GTCATCAAGGCAGAACAGCCAGATGGGTTAATTCTGGGCATGGGTGGCCAGACAGCTCTG
AACTGTGGAGTGGAACTATTCAAGAGAGGTGTGCTCAAGGAATATGGTGTGAAAGTCCTG
GGAACTTCAGTTGAGTCCATTATGGCTACGGAAGACAGGCAGCTGTTTTCAGATAAACTA
AATGAGATCAATGAAAAGATTGCTCCAAGTTTTGCAGTGGAATCGATTGAGGATGCACTG
AAGGCAGCAGACACCATTGGCTACCCAGTGATGATCCGTTCCGCCTATGCACTGGGTGGG
TTAGGCTCAGGCATCTGTCCCAACAGAGAGACTTTGATGGACCTCAGCACAAAGGCCTTT
GCTATGACCAACCAAATTCTGGTGGAGAAGTCAGTGACAGGTTGGAAAGAAATAGAATAT
GAAGTGGTTCGAGATGCTGATGACAATTGTGTCACTGTCTGTAACATGGAAAATGTTGAT
GCCATGGGTGTTCACACAGGTGACTCAGTTGTTGTGGCTCCTGCCCAGACACTCTCCAAT
GCCGAGTTTCAGATGTTGAGACGTACTTCAATCAATGTTGTTCGCCACTTGGGCATTGTG
GGTGAATGCAACATTCAGTTTGCCCTTCATCCTACCTCAATGGAATACTGCATCATTGAA
GTGAATGCCAGACTGTCCCGAAGCTCTGCTCTGGCCTCAAAAGCCACTGGCTACCCATTG
GCATTCATTGCTGCAAAGATTGCCCTAGGAATCCCACTTCCAGAAATTAAGAACGTCGTA
TCCGGGAAGACATCAGCCTGTTTTGAACCTAGCCTGGATTACATGGTCACCAAGATTCCC
CGCTGGGATCTTGACCGTTTTCATGGAACATCTAGCCGAATTGGTAGCTCTATGAAAAGT
GTAGGAGAGGTCATGGCTATTGGTCGTACCTTTGAGGAGAGTTTCCAGAAAGCTTTACGG
ATGTGCCACCCATCTATAGAAGGTTTCACTCCCCGTCTCCCAATGAACAAAGAATGGCCA
TCTAATTTAGATCTTAGAAAAGAGTTGTCTGAACCAAGCAGCACGCGTATCTATGCCATT
GCCAAGGCCATTGATGACAACATGTCCCTTGATGAGATTGAGAAGCTCACATACATTGAC
AAGTGGTTTTTGTATAAGATGCGTGATATTTTAAACATGGAAAAGACACTGAAAGGCCTC
AACAGTGAGTCCATGACAGAAGAAACCCTGAAAAGGGCAAAGGAGATTGGGTTCTCAGAT
AAGCAGATTTCAAAATGCCTTGGGCTCACTGAGGCCCAGACAAGGGAGCTGAGGTTAAAG
AAAAACATCCACCCTTGGGTTAAACAGATTGATACACTGGCTGCAGAATACCCATCAGTA
ACAAACTATCTCTATGTTACCTACAATGGTCAGGAGCATGATGTCAATTTTGATGACCAT
GGAATGATGGTGCTAGGCTGTGGTCCATATCACATTGGCAGCAGTGTGGAATTTGATTGG
TGTGCTGTCTCTAGTATCCGCACACTGCGTCAACTTGGCAAGAAGACGGTGGTGGTGAAT
TGCAATCCTGAGACTGTGAGCACAGACTTTGATGAGTGTGACAAACTGTACTTTGAAGAG
TTGTCCTTGGAGAGAATCCTAGACATCTACCATCAGGAGGCATGTGGTGGCTGCATCATA
TCAGTTGGAGGCCAGATTCCAAACAACCTGGCAGTTCCTCTATACAAGAATGGTGTCAAG
ATCATGGGCACAAGCCCCCTGCAGATCGACAGGGCTGAGGATCGCTCCATCTTCTCAGCT
GTCTTGGATGAGCTGAAGGTGGCTCAGGCACCTTGGAAAGCTGTTAATACTTTGAATGAA
GCACTGGAATTTGCAAAGTCTGTGGACTACCCCTGCTTGTTGAGGCCTTCCTATGTTTTG
AGTGGGTCTGCTATGAATGTGGTATTCTCTGAGGATGAGATGAAAAAATTCCTAGAAGAG
GCGACTAGAGTTTCTCAGGAGCACCCAGTGGTGCTGACAAAATTTGTTGAAGGGGCCCGA
GAAGTAGAAATGGACGCTGTTGGCAAAGATGGAAGGGTTATCTCTCATGCCATCTCTGAA
CATGTTGAAGATGCAGGTGTCCACTCGGGAGATGCCACTCTGATGCTGCCCACACAAACC
ATCAGCCAAGGGGCCATTGAAAAGGTGAAGGATGCTACCCGGAAGATTGCAAAGGCTTTT
GCCATCTCTGGTCCATTCAACGTCCAATTTCTTGTCAAAGGAAATGATGTCTTGGTGATT
GAGTGTAACTTGAGAGCTTCTCGATCCTTCCCCTTTGTTTCCAAGACTCTTGGGGTTGAC
TTCATTGATGTGGCCACCAAGGTGATGATTGGAGAGAATGTTGATGAGAAACATCTTCCA
ACATTGGACCATCCCATAATTCCTGCTGACTATGTTGCAATTAAGGCTCCCATGTTTTCC
TGGCCCCGGTTGAGGGATGCTGACCCCATTCTGAGATGTGAGATGGCTTCCACTGGAGAG
GTGGCTTGCTTTGGTGAAGGTATTCATACAGCCTTCCTAAAGGCAATGCTTTCCACAGGA
TTTAAGATACCCCAGAAAGGCATCCTGATAGGCATCCAGCAATCATTCCGGCCAAGATTC
CTTGGTGTGGCTGAACAATTACACAATGAAGGTTTCAAGCTGTTTGCCACGGAAGCCACA
TCAGACTGGCTCAACGCCAACAATGTCCCTGCCACCCCAGTGGCATGGCCGTCTCAAGAA
GGACAGAATCCCAGCCTCTCTTCCATCAGAAAATTGATTAGAGATGGCAGCATTGACCTA
GTGATTAACCTTCCCAACAACAACACTAAATTTGTCCATGATAATTATGTGATTCGGAGG
ACAGCTGTTGATAGTGGAATCCCTCTCCTCACTAATTTTCAGGTGACCAAACTTTTTGCT
GAAGCTGTGCAGAAATCTCGCAAGGTGGACTCCAAGAGTCTTTTCCACTACAGGCAGTAC
AGTGCTGGAAAAGCAGCATAG
Chromosome Location
2
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDP31327
UniProtKB Entry NameCPSM_HUMAN
HGNC IDHGNC:2323
General References
  1. Haraguchi Y, Uchino T, Takiguchi M, Endo F, Mori M, Matsuda I: Cloning and sequence of a cDNA encoding human carbamyl phosphate synthetase I: molecular analysis of hyperammonemia. Gene. 1991 Nov 15;107(2):335-40. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB06775Carglumic acidapprovedyesallosteric modulatorDetails
DB11118AmmoniaapprovedunknownsubstrateDetails