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

Gene name
CPS1, CPSASE1, PHN
Definition
carbamoyl-phosphate synthase 1, mitochondrial (EC:6.3.4.16)
Orthology
K01948  
carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16]
Organism
hsa  Homo sapiens (human)
Pathway
Alanine, aspartate and glutamate metabolism
Arginine and proline metabolism
Nitrogen metabolism
Metabolic pathways
Carbon metabolism
Biosynthesis of amino acids
Module
Urea cycle
Disease
H00164  
Carbamoyl phosphate synthetase I deficiency
Drug target
Carglumic acid: 
Class
Metabolism; Overview; Carbon metabolism [PATH:hsa01200]
Metabolism; Overview; Biosynthesis of amino acids [PATH:hsa01230]
Metabolism; Energy metabolism; Nitrogen metabolism [PATH:hsa00910]
Metabolism; Amino acid metabolism; Alanine, aspartate and glutamate metabolism [PATH:hsa00250]
Metabolism; Amino acid metabolism; Arginine and proline metabolism [PATH:hsa00330]
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif Motif
Other DBs
NCBI-GI: 
NCBI-GeneID: 
OMIM: 
HGNC: 
HPRD: 
Ensembl: 
Vega: 
UniProt: 
Structure
PDB: 

Jmol
Position
2q35
AA seq 1506 aa AA seqDB search
MPQIIKMTRILTAFKVVRTLKTGFGFTNVTAHQKWKFSRPGIRLLSVKAQTAHIVLEDGT
KMKGYSFGHPSSVAGEVVFNTGLGGYPEAITDPAYKGQILTMANPIIGNGGAPDTTALDE
LGLSKYLESNGIKVSGLLVLDYSKDYNHWLATKSLGQWLQEEKVPAIYGVDTRMLTKIIR
DKGTMLGKIEFEGQPVDFVDPNKQNLIAEVSTKDVKVYGKGNPTKVVAVDCGIKNNVIRL
LVKRGAEVHLVPWNHDFTKMEYDGILIAGGPGNPALAEPLIQNVRKILESDRKEPLFGIS
TGNLITGLAAGAKTYKMSMANRGQNQPVLNITNKQAFITAQNHGYALDNTLPAGWKPLFV
NVNDQTNEGIMHESKPFFAVQFHPEVTPGPIDTEYLFDSFFSLIKKGKATTITSVLPKPA
LVASRVEVSKVLILGSGGLSIGQAGEFDYSGSQAVKAMKEENVKTVLMNPNIASVQTNEV
GLKQADTVYFLPITPQFVTEVIKAEQPDGLILGMGGQTALNCGVELFKRGVLKEYGVKVL
GTSVESIMATEDRQLFSDKLNEINEKIAPSFAVESIEDALKAADTIGYPVMIRSAYALGG
LGSGICPNRETLMDLSTKAFAMTNQILVEKSVTGWKEIEYEVVRDADDNCVTVCNMENVD
AMGVHTGDSVVVAPAQTLSNAEFQMLRRTSINVVRHLGIVGECNIQFALHPTSMEYCIIE
VNARLSRSSALASKATGYPLAFIAAKIALGIPLPEIKNVVSGKTSACFEPSLDYMVTKIP
RWDLDRFHGTSSRIGSSMKSVGEVMAIGRTFEESFQKALRMCHPSIEGFTPRLPMNKEWP
SNLDLRKELSEPSSTRIYAIAKAIDDNMSLDEIEKLTYIDKWFLYKMRDILNMEKTLKGL
NSESMTEETLKRAKEIGFSDKQISKCLGLTEAQTRELRLKKNIHPWVKQIDTLAAEYPSV
TNYLYVTYNGQEHDVNFDDHGMMVLGCGPYHIGSSVEFDWCAVSSIRTLRQLGKKTVVVN
CNPETVSTDFDECDKLYFEELSLERILDIYHQEACGGCIISVGGQIPNNLAVPLYKNGVK
IMGTSPLQIDRAEDRSIFSAVLDELKVAQAPWKAVNTLNEALEFAKSVDYPCLLRPSYVL
SGSAMNVVFSEDEMKKFLEEATRVSQEHPVVLTKFVEGAREVEMDAVGKDGRVISHAISE
HVEDAGVHSGDATLMLPTQTISQGAIEKVKDATRKIAKAFAISGPFNVQFLVKGNDVLVI
ECNLRASRSFPFVSKTLGVDFIDVATKVMIGENVDEKHLPTLDHPIIPADYVAIKAPMFS
WPRLRDADPILRCEMASTGEVACFGEGIHTAFLKAMLSTGFKIPQKGILIGIQQSFRPRF
LGVAEQLHNEGFKLFATEATSDWLNANNVPATPVAWPSQEGQNPSLSSIRKLIRDGSIDL
VINLPNNNTKFVHDNYVIRRTAVDSGIPLLTNFQVTKLFAEAVQKSRKVDSKSLFHYRQY
SAGKAA
NT seq 4521 nt NT seq  +upstreamnt  +downstreamnt
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

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