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
- FunctionsATP binding / calcium ion binding / carbamoyl-phosphate synthase (ammonia) activity / carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity / endopeptidase activity / glutamate binding / modified amino acid binding / phospholipid bindingProcesses'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 cycleComponentscytoplasm / 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
Resource Link UniProtKB ID P31327 UniProtKB Entry Name CPSM_HUMAN HGNC ID HGNC:2323 - General References
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