KEGG   Homo sapiens (human): 1373Help
Entry
1373              CDS       T01001                                 

Gene name
CPS1, CPSASE1, PHN
Definition
(RefSeq) carbamoyl-phosphate synthase 1 (EC:6.3.4.16)
  KO
K01948  
carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16]
Organism
hsa  Homo sapiens (human)
Pathway
Arginine biosynthesis
Alanine, aspartate and glutamate metabolism
Nitrogen metabolism
Metabolic pathways
Carbon metabolism
Biosynthesis of amino acids
Module
Urea cycle
Disease
H00164  
Carbamoyl phosphate synthetase I deficiency
H01398  
Primary hyperammonemic disorders
Drug target
Carglumic acid: 
Brite
KEGG Orthology (KO) [BR:hsa00001]
 Metabolism
  Overview
   01200 Carbon metabolism
    1373 (CPS1)
   01230 Biosynthesis of amino acids
    1373 (CPS1)
  Energy metabolism
   00910 Nitrogen metabolism
    1373 (CPS1)
  Amino acid metabolism
   00250 Alanine, aspartate and glutamate metabolism
    1373 (CPS1)
   00220 Arginine biosynthesis
    1373 (CPS1)
Enzymes [BR:hsa01000]
 6. Ligases
  6.3  Forming carbon-nitrogen bonds
   6.3.4  Other carbon-nitrogen ligases
    6.3.4.16  carbamoyl-phosphate synthase (ammonia)
     1373 (CPS1)
Peptidases [BR:hsa01002]
 Cysteine Peptidases
  Family C26: gamma-glutamyl hydrolase family
   1373 (CPS1)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif Motif
Other DBs
NCBI-ProteinID: 
NCBI-GeneID: 
OMIM: 
HGNC: 
HPRD: 
Ensembl: 
Vega: 
UniProt: 
LinkDB All DBs
Structure
PDB: 

Jmol
Position
2q35
AA seq 1500 aa AA seqDB search
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
NT seq 4503 nt NT seq  +upstreamnt  +downstreamnt
atgacgaggattttgacagctttcaaagtggtgaggacactgaagactggttttggcttt
accaatgtgactgcacaccaaaaatggaaattttcaagacctggcatcaggctcctttct
gtcaaggcacagacagcacacattgtcctggaagatggaactaagatgaaaggttactcc
tttggccatccatcctctgttgctggtgaagtggtttttaatactggcctgggagggtac
ccagaagctattactgaccctgcctacaaaggacagattctcacaatggccaaccctatt
attgggaatggtggagctcctgatactactgctctggatgaactgggacttagcaaatat
ttggagtctaatggaatcaaggtttcaggtttgctggtgctggattatagtaaagactac
aaccactggctggctaccaagagtttagggcaatggctacaggaagaaaaggttcctgca
atttatggagtggacacaagaatgctgactaaaataattcgggataagggtaccatgctt
gggaagattgaatttgaaggtcagcctgtggattttgtggatccaaataaacagaatttg
attgctgaggtttcaaccaaggatgtcaaagtgtacggcaaaggaaaccccacaaaagtg
gtagctgtagactgtgggattaaaaacaatgtaatccgcctgctagtaaagcgaggagct
gaagtgcacttagttccctggaaccatgatttcaccaagatggagtatgatgggattttg
atcgcgggaggaccggggaacccagctcttgcagaaccactaattcagaatgtcagaaag
attttggagagtgatcgcaaggagccattgtttggaatcagtacaggaaacttaataaca
ggattggctgctggtgccaaaacctacaagatgtccatggccaacagagggcagaatcag
cctgttttgaatatcacaaacaaacaggctttcattactgctcagaatcatggctatgcc
ttggacaacaccctccctgctggctggaaaccactttttgtgaatgtcaacgatcaaaca
aatgaggggattatgcatgagagcaaacccttcttcgctgtgcagttccacccagaggtc
accccggggccaatagacactgagtacctgtttgattcctttttctcactgataaagaaa
ggaaaagctaccaccattacatcagtcttaccgaagccagcactagttgcatctcgggtt
gaggtttccaaagtccttattctaggatcaggaggtctgtccattggtcaggctggagaa
tttgattactcaggatctcaagctgtaaaagccatgaaggaagaaaatgtcaaaactgtt
ctgatgaacccaaacattgcatcagtccagaccaatgaggtgggcttaaagcaagcggat
actgtctactttcttcccatcacccctcagtttgtcacagaggtcatcaaggcagaacag
ccagatgggttaattctgggcatgggtggccagacagctctgaactgtggagtggaacta
ttcaagagaggtgtgctcaaggaatatggtgtgaaagtcctgggaacttcagttgagtcc
attatggctacggaagacaggcagctgttttcagataaactaaatgagatcaatgaaaag
attgctccaagttttgcagtggaatcgattgaggatgcactgaaggcagcagacaccatt
ggctacccagtgatgatccgttccgcctatgcactgggtgggttaggctcaggcatctgt
cccaacagagagactttgatggacctcagcacaaaggcctttgctatgaccaaccaaatt
ctggtggagaagtcagtgacaggttggaaagaaatagaatatgaagtggttcgagatgct
gatgacaattgtgtcactgtctgtaacatggaaaatgttgatgccatgggtgttcacaca
ggtgactcagttgttgtggctcctgcccagacactctccaatgccgagtttcagatgttg
agacgtacttcaatcaatgttgttcgccacttgggcattgtgggtgaatgcaacattcag
tttgcccttcatcctacctcaatggaatactgcatcattgaagtgaatgccagactgtcc
cgaagctctgctctggcctcaaaagccactggctacccattggcattcattgctgcaaag
attgccctaggaatcccacttccagaaattaagaacgtcgtatccgggaagacatcagcc
tgttttgaacctagcctggattacatggtcaccaagattccccgctgggatcttgaccgt
tttcatggaacatctagccgaattggtagctctatgaaaagtgtaggagaggtcatggct
attggtcgtacctttgaggagagtttccagaaagctttacggatgtgccacccatctata
gaaggtttcactccccgtctcccaatgaacaaagaatggccatctaatttagatcttaga
aaagagttgtctgaaccaagcagcacgcgtatctatgccattgccaaggccattgatgac
aacatgtcccttgatgagattgagaagctcacatacattgacaagtggtttttgtataag
atgcgtgatattttaaacatggaaaagacactgaaaggcctcaacagtgagtccatgaca
gaagaaaccctgaaaagggcaaaggagattgggttctcagataagcagatttcaaaatgc
cttgggctcactgaggcccagacaagggagctgaggttaaagaaaaacatccacccttgg
gttaaacagattgatacactggctgcagaatacccatcagtaacaaactatctctatgtt
acctacaatggtcaggagcatgatgtcaattttgatgaccatggaatgatggtgctaggc
tgtggtccatatcacattggcagcagtgtggaatttgattggtgtgctgtctctagtatc
cgcacactgcgtcaacttggcaagaagacggtggtggtgaattgcaatcctgagactgtg
agcacagactttgatgagtgtgacaaactgtactttgaagagttgtccttggagagaatc
ctagacatctaccatcaggaggcatgtggtggctgcatcatatcagttggaggccagatt
ccaaacaacctggcagttcctctatacaagaatggtgtcaagatcatgggcacaagcccc
ctgcagatcgacagggctgaggatcgctccatcttctcagctgtcttggatgagctgaag
gtggctcaggcaccttggaaagctgttaatactttgaatgaagcactggaatttgcaaag
tctgtggactacccctgcttgttgaggccttcctatgttttgagtgggtctgctatgaat
gtggtattctctgaggatgagatgaaaaaattcctagaagaggcgactagagtttctcag
gagcacccagtggtgctgacaaaatttgttgaaggggcccgagaagtagaaatggacgct
gttggcaaagatggaagggttatctctcatgccatctctgaacatgttgaagatgcaggt
gtccactcgggagatgccactctgatgctgcccacacaaaccatcagccaaggggccatt
gaaaaggtgaaggatgctacccggaagattgcaaaggcttttgccatctctggtccattc
aacgtccaatttcttgtcaaaggaaatgatgtcttggtgattgagtgtaacttgagagct
tctcgatccttcccctttgtttccaagactcttggggttgacttcattgatgtggccacc
aaggtgatgattggagagaatgttgatgagaaacatcttccaacattggaccatcccata
attcctgctgactatgttgcaattaaggctcccatgttttcctggccccggttgagggat
gctgaccccattctgagatgtgagatggcttccactggagaggtggcttgctttggtgaa
ggtattcatacagccttcctaaaggcaatgctttccacaggatttaagataccccagaaa
ggcatcctgataggcatccagcaatcattccggccaagattccttggtgtggctgaacaa
ttacacaatgaaggtttcaagctgtttgccacggaagccacatcagactggctcaacgcc
aacaatgtccctgccaccccagtggcatggccgtctcaagaaggacagaatcccagcctc
tcttccatcagaaaattgattagagatggcagcattgacctagtgattaaccttcccaac
aacaacactaaatttgtccatgataattatgtgattcggaggacagctgttgatagtgga
atccctctcctcactaattttcaggtgaccaaactttttgctgaagctgtgcagaaatct
cgcaaggtggactccaagagtcttttccactacaggcagtacagtgctggaaaagcagca
tag

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