KEGG   PATHWAY: ko00240Help
Entry
ko00240                     Pathway                                

Name
Pyrimidine metabolism
Class
Metabolism; Nucleotide metabolism
BRITE hierarchy
Pathway map
ko00240  Pyrimidine metabolism
ko00240

Ortholog table
Module
M00046  Pyrimidine degradation, uracil => beta-alanine, thymine => 3-aminoisobutanoate [PATH:ko00240]
M00051  Uridine monophosphate biosynthesis, glutamine (+ PRPP) => UMP [PATH:ko00240]
M00052  Pyrimidine ribonucleotide biosynthesis, UMP => UDP/UTP,CDP/CTP [PATH:ko00240]
M00053  Pyrimidine deoxyribonuleotide biosynthesis, CDP/CTP => dCDP/dCTP,dTDP/dTTP [PATH:ko00240]
Disease
H00193  Dihydropyrimidine dehydrogenase deficiency
H00198  Orotic aciduria
H00199  Dihydropyrimidinase deficiency
H00200  Beta-ureidopropionase deficiency
H00469  Mitochondrial DNA depletion syndrome (MDS)
H00494  Desbuquois syndrome
H00674  Anemia due to disorders of nucleotide metabolism
H00824  Calcification of joints and arteries
H01118  Progressive external ophthalmoplegia (PEO)
H01376  Acrofacial dysostosis
H01390  Mitochondrial neurogastrointestinal encephalomyopathy
Drug
D00323  Flucytosine (JP17/USP/INN)
D00391  Trifluridine (JAN/USP/INN)
D00488  Pyrimethamine (JAN/USP/INN)
D02487  Proguanil hydrochloride (JAN)
D03034  Azelaic acid (USAN/INN)
D03771  Diaveridine (USAN/INN)
D08428  Proguanil (INN)
Other DBs
BSID: 309
GO: 0006220
Orthology
K11540  carbamoyl-phosphate synthase / aspartate carbamoyltransferase / dihydroorotase [EC:3.5.2.3 2.1.3.2 6.3.5.5]
K11541  carbamoyl-phosphate synthase / aspartate carbamoyltransferase [EC:2.1.3.2 6.3.5.5]
K01954  carbamoyl-phosphate synthase [EC:6.3.5.5]
K01955  carbamoyl-phosphate synthase large subunit [EC:6.3.5.5]
K01956  carbamoyl-phosphate synthase small subunit [EC:6.3.5.5]
K00609  aspartate carbamoyltransferase catalytic subunit [EC:2.1.3.2]
K00610  aspartate carbamoyltransferase regulatory subunit
K00608  aspartate carbamoyltransferase [EC:2.1.3.2]
K01465  dihydroorotase [EC:3.5.2.3]
K17828  dihydroorotate dehydrogenase (NAD+) catalytic subunit [EC:1.3.1.14]
K02823  dihydroorotate dehydrogenase electron transfer subunit
K00226  dihydroorotate dehydrogenase (fumarate) [EC:1.3.98.1]
K00254  dihydroorotate dehydrogenase [EC:1.3.5.2]
K13421  uridine monophosphate synthetase [EC:4.1.1.23 2.4.2.10]
K00762  orotate phosphoribosyltransferase [EC:2.4.2.10]
K01591  orotidine-5'-phosphate decarboxylase [EC:4.1.1.23]
K13800  UMP-CMP kinase [EC:2.7.4.14]
K13809  UMP-CMP kinase 2, mitochondrial [EC:2.7.4.14]
K09903  uridylate kinase [EC:2.7.4.22]
K00940  nucleoside-diphosphate kinase [EC:2.7.4.6]
K18533  adenylate/nucleoside-diphosphate kinase [EC:2.7.4.6 2.7.4.3]
K00962  polyribonucleotide nucleotidyltransferase [EC:2.7.7.8]
K01510  apyrase [EC:3.6.1.5]
K14641  apyrase [EC:3.6.1.5]
K14642  golgi apyrase [EC:3.6.1.5]
K12304  soluble calcium-activated nucleotidase 1 [EC:3.6.1.6]
K12305  ectonucleoside triphosphate diphosphohydrolase 4 [EC:3.6.1.6]
K01511  ectonucleoside triphosphate diphosphohydrolase 5/6 [EC:3.6.1.6]
K01937  CTP synthase [EC:6.3.4.2]
K00945  CMP/dCMP kinase [EC:2.7.4.25]
K13799  pantoate ligase / CMP/dCMP kinase [EC:2.7.4.25 6.3.2.1]
K03040  DNA-directed RNA polymerase subunit alpha [EC:2.7.7.6]
K03043  DNA-directed RNA polymerase subunit beta [EC:2.7.7.6]
K03046  DNA-directed RNA polymerase subunit beta' [EC:2.7.7.6]
K13797  DNA-directed RNA polymerase subunit beta-beta' [EC:2.7.7.6]
K03048  DNA-directed RNA polymerase subunit delta
K03060  DNA-directed RNA polymerase subunit omega [EC:2.7.7.6]
K03042  DNA-directed RNA polymerase subunit A" [EC:2.7.7.6]
K03041  DNA-directed RNA polymerase subunit A' [EC:2.7.7.6]
K03044  DNA-directed RNA polymerase subunit B' [EC:2.7.7.6]
K03045  DNA-directed RNA polymerase subunit B" [EC:2.7.7.6]
K13798  DNA-directed RNA polymerase subunit B [EC:2.7.7.6]
K03053  DNA-directed RNA polymerase subunit H [EC:2.7.7.6]
K03055  DNA-directed RNA polymerase subunit K [EC:2.7.7.6]
K03058  DNA-directed RNA polymerase subunit N [EC:2.7.7.6]
K03047  DNA-directed RNA polymerase subunit D [EC:2.7.7.6]
K03049  DNA-directed RNA polymerase subunit E' [EC:2.7.7.6]
K03050  DNA-directed RNA polymerase subunit E" [EC:2.7.7.6]
K03056  DNA-directed RNA polymerase subunit L [EC:2.7.7.6]
K03059  DNA-directed RNA polymerase subunit P [EC:2.7.7.6]
K03051  DNA-directed RNA polymerase subunit F [EC:2.7.7.6]
K03052  DNA-directed RNA polymerase subunit G [EC:2.7.7.6]
K03054  DNA-directed RNA polymerase subunit I [EC:2.7.7.6]
K02999  DNA-directed RNA polymerase I subunit RPA1 [EC:2.7.7.6]
K03002  DNA-directed RNA polymerase I subunit RPA2 [EC:2.7.7.6]
K03000  DNA-directed RNA polymerase I subunit RPA12
K03001  DNA-directed RNA polymerase I subunit RPA14
K03003  DNA-directed RNA polymerase I subunit RPA34
K03004  DNA-directed RNA polymerase I subunit RPA43
K03005  DNA-directed RNA polymerase I subunit RPA49
K03006  DNA-directed RNA polymerase II subunit RPB1 [EC:2.7.7.6]
K03010  DNA-directed RNA polymerase II subunit RPB2 [EC:2.7.7.6]
K03011  DNA-directed RNA polymerase II subunit RPB3
K03012  DNA-directed RNA polymerase II subunit RPB4
K03013  DNA-directed RNA polymerases I, II, and III subunit RPABC1
K03014  DNA-directed RNA polymerases I, II, and III subunit RPABC2
K03015  DNA-directed RNA polymerase II subunit RPB7
K03016  DNA-directed RNA polymerases I, II, and III subunit RPABC3
K03017  DNA-directed RNA polymerase II subunit RPB9
K03007  DNA-directed RNA polymerases I, II, and III subunit RPABC5
K03008  DNA-directed RNA polymerase II subunit RPB11
K03009  DNA-directed RNA polymerases I, II, and III subunit RPABC4
K03018  DNA-directed RNA polymerase III subunit RPC1 [EC:2.7.7.6]
K03021  DNA-directed RNA polymerase III subunit RPC2 [EC:2.7.7.6]
K03023  DNA-directed RNA polymerase III subunit RPC3
K03026  DNA-directed RNA polymerase III subunit RPC4
K14721  DNA-directed RNA polymerase III subunit RPC5
K03027  DNA-directed RNA polymerases I and III subunit RPAC1
K03019  DNA-directed RNA polymerase III subunit RPC11
K03020  DNA-directed RNA polymerases I and III subunit RPAC2
K03022  DNA-directed RNA polymerase III subunit RPC8
K03024  DNA-directed RNA polymerase III subunit RPC7
K03025  DNA-directed RNA polymerase III subunit RPC6
K02335  DNA polymerase I [EC:2.7.7.7]
K02337  DNA polymerase III subunit alpha [EC:2.7.7.7]
K03763  DNA polymerase III subunit alpha, Gram-positive type [EC:2.7.7.7]
K02338  DNA polymerase III subunit beta [EC:2.7.7.7]
K02343  DNA polymerase III subunit gamma/tau [EC:2.7.7.7]
K02340  DNA polymerase III subunit delta [EC:2.7.7.7]
K02341  DNA polymerase III subunit delta' [EC:2.7.7.7]
K02342  DNA polymerase III subunit epsilon [EC:2.7.7.7]
K14159  ribonuclease HI / DNA polymerase III subunit epsilon [EC:2.7.7.7 3.1.26.4]
K02345  DNA polymerase III subunit theta [EC:2.7.7.7]
K02339  DNA polymerase III subunit chi [EC:2.7.7.7]
K02344  DNA polymerase III subunit psi [EC:2.7.7.7]
K02319  DNA polymerase, archaea type [EC:2.7.7.7]
K02322  DNA polymerase II large subunit [EC:2.7.7.7]
K02323  DNA polymerase II small subunit [EC:2.7.7.7]
K02320  DNA polymerase alpha subunit A [EC:2.7.7.7]
K02321  DNA polymerase alpha subunit B
K02684  DNA primase small subunit [EC:2.7.7.-]
K02685  DNA primase large subunit [EC:2.7.7.-]
K02327  DNA polymerase delta subunit 1 [EC:2.7.7.7]
K02328  DNA polymerase delta subunit 2
K03504  DNA polymerase delta subunit 3
K03505  DNA polymerase delta subunit 4
K02324  DNA polymerase epsilon subunit 1 [EC:2.7.7.7]
K02325  DNA polymerase epsilon subunit 2 [EC:2.7.7.7]
K02326  DNA polymerase epsilon subunit 3 [EC:2.7.7.7]
K03506  DNA polymerase epsilon subunit 4 [EC:2.7.7.7]
K01494  dCTP deaminase [EC:3.5.4.13]
K00876  uridine kinase [EC:2.7.1.48]
K21057  cytidine kinase [EC:2.7.1.213]
K22026  nucleoside kinase [EC:2.7.1.- 2.7.1.213 2.7.1.73]
K01081  5'-nucleotidase [EC:3.1.3.5]
K19970  5'-nucleotidase [EC:3.1.3.5]
K11751  5'-nucleotidase / UDP-sugar diphosphatase [EC:3.6.1.45 3.1.3.5]
K03787  5'-nucleotidase [EC:3.1.3.5]
K08723  5'-nucleotidase [EC:3.1.3.5]
K20881  5'-nucleotidase [EC:3.1.3.5]
K00757  uridine phosphorylase [EC:2.4.2.3]
K01240  uridine nucleosidase [EC:3.2.2.3]
K00761  uracil phosphoribosyltransferase [EC:2.4.2.9]
K02825  pyrimidine operon attenuation protein / uracil phosphoribosyltransferase [EC:2.4.2.9]
K00207  dihydropyrimidine dehydrogenase (NADP+) [EC:1.3.1.2]
K17722  dihydropyrimidine dehydrogenase (NAD+) subunit PreT [EC:1.3.1.1]
K17723  dihydropyrimidine dehydrogenase (NAD+) subunit PreA [EC:1.3.1.1]
K01464  dihydropyrimidinase [EC:3.5.2.2]
K01431  beta-ureidopropionase [EC:3.5.1.6]
K06016  beta-ureidopropionase / N-carbamoyl-L-amino-acid hydrolase [EC:3.5.1.87 3.5.1.6]
K19794  barbiturase [EC:3.5.2.1]
K19795  ureidomalonase [EC:3.5.1.95]
K09018  pyrimidine oxygenase [EC:1.14.99.46]
K09024  flavin reductase [EC:1.5.1.-]
K09020  ureidoacrylate peracid hydrolase [EC:3.5.1.110]
K09021  aminoacrylate peracid reductase
K09023  aminoacrylate hydrolase [EC:3.5.1.-]
K16066  3-hydroxy acid dehydrogenase / malonic semialdehyde reductase [EC:1.1.1.- 1.1.1.381]
K09019  3-hydroxypropanoate dehydrogenase [EC:1.1.1.-]
K00756  pyrimidine-nucleoside phosphorylase [EC:2.4.2.2]
K09913  purine/pyrimidine-nucleoside phosphorylase [EC:2.4.2.2 2.4.2.1]
K10213  ribosylpyrimidine nucleosidase [EC:3.2.2.8]
K06966  pyrimidine/purine-5'-nucleotide nucleosidase [EC:3.2.2.- 3.2.2.10]
K01485  cytosine deaminase [EC:3.5.4.1]
K01513  ectonucleotide pyrophosphatase/phosphodiesterase family member 1/3 [EC:3.6.1.9 3.1.4.1]
K04765  nucleoside triphosphate diphosphatase [EC:3.6.1.9]
K00527  ribonucleoside-triphosphate reductase (thioredoxin) [EC:1.17.4.2]
K21636  ribonucleoside-triphosphate reductase (formate) [EC:1.1.98.6]
K00524  ribonucleotide reductase, class II [EC:1.17.4.1]
K00525  ribonucleoside-diphosphate reductase alpha chain [EC:1.17.4.1]
K00526  ribonucleoside-diphosphate reductase beta chain [EC:1.17.4.1]
K10807  ribonucleoside-diphosphate reductase subunit M1 [EC:1.17.4.1]
K10808  ribonucleoside-diphosphate reductase subunit M2 [EC:1.17.4.1]
K16904  dCTP diphosphatase [EC:3.6.1.12]
K09887  dCTP deaminase (dUMP-forming) [EC:3.5.4.30]
K01520  dUTP pyrophosphatase [EC:3.6.1.23]
K00560  thymidylate synthase [EC:2.1.1.45]
K13998  dihydrofolate reductase / thymidylate synthase [EC:2.1.1.45 1.5.1.3]
K03465  thymidylate synthase (FAD) [EC:2.1.1.148]
K01489  cytidine deaminase [EC:3.5.4.5]
K00758  thymidine phosphorylase [EC:2.4.2.4]
K03783  purine-nucleoside phosphorylase [EC:2.4.2.1]
K03784  purine-nucleoside phosphorylase [EC:2.4.2.1]
K00893  deoxycitidine kinase [EC:2.7.1.74]
K15519  deoxyadenosine/deoxycytidine kinase [EC:2.7.1.74 2.7.1.76]
K08722  5'-deoxynucleotidase [EC:3.1.3.89]
K01493  dCMP deaminase [EC:3.5.4.12]
K19796  dCMP hydroxymethylase [EC:2.1.2.8]
K19797  T-even-induced deoxynucleotide kinase [EC:2.7.4.12]
K18942  T4virus DNA polymerase [EC:2.7.7.7]
K08728  nucleoside deoxyribosyltransferase [EC:2.4.2.6]
K00857  thymidine kinase [EC:2.7.1.21]
K00943  dTMP kinase [EC:2.7.4.9]
K01119  2',3'-cyclic-nucleotide 2'-phosphodiesterase / 3'-nucleotidase [EC:3.1.3.6 3.1.4.16]
K08693  2',3'-cyclic-nucleotide 2'-phosphodiesterase / 3'-nucleotidase / 5'-nucleotidase [EC:3.1.3.5 3.1.3.6 3.1.4.16]
K01518  bis(5'-nucleosidyl)-tetraphosphatase [EC:3.6.1.17]
K16328  pseudouridine kinase [EC:2.7.1.83]
K16329  pseudouridylate synthase [EC:4.2.1.70]
K16330  pseudouridylate synthase / pseudouridine kinase [EC:2.7.1.83 4.2.1.70]
Compound
C00015  UDP
C00029  UDP-glucose
C00039  DNA
C00046  RNA
C00055  CMP
C00063  CTP
C00064  L-Glutamine
C00075  UTP
C00086  Urea
C00099  beta-Alanine
C00105  UMP
C00106  Uracil
C00112  CDP
C00119  5-Phospho-alpha-D-ribose 1-diphosphate
C00169  Carbamoyl phosphate
C00178  Thymine
C00214  Thymidine
C00222  3-Oxopropanoate
C00239  dCMP
C00295  Orotate
C00299  Uridine
C00337  (S)-Dihydroorotate
C00363  dTDP
C00364  dTMP
C00365  dUMP
C00380  Cytosine
C00383  Malonate
C00429  5,6-Dihydrouracil
C00438  N-Carbamoyl-L-aspartate
C00458  dCTP
C00459  dTTP
C00460  dUTP
C00475  Cytidine
C00526  Deoxyuridine
C00672  2-Deoxy-D-ribose 1-phosphate
C00705  dCDP
C00813  Barbiturate
C00881  Deoxycytidine
C01013  3-Hydroxypropanoate
C01103  Orotidine 5'-phosphate
C01168  Pseudouridine 5'-phosphate
C01205  (R)-3-Amino-2-methylpropanoate
C01346  dUDP
C01368  3'-UMP
C01563  Carbamate
C02067  Pseudouridine
C02170  Methylmalonate
C02354  2',3'-Cyclic CMP
C02355  2',3'-Cyclic UMP
C02376  5-Methylcytosine
C02642  3-Ureidopropionate
C03997  5-Hydroxymethyldeoxycytidylate
C05281  5-Methylbarbiturate
C05822  3'-CMP
C06198  P1,P4-Bis(5'-uridyl) tetraphosphate
C11038  2'-Deoxy-5-hydroxymethylcytidine-5'-diphosphate
C11039  2'-Deoxy-5-hydroxymethylcytidine-5'-triphosphate
C15607  3-Oxo-3-ureidopropanoate
C20231  (Z)-3-Ureidoacrylate peracid
C20249  (Z)-3-Peroxyaminoacrylate
C20253  Aminoacrylate
C20254  Ureidoacrylate
C20846  3-Oxo-3-ureidoisobutyrate
C21028  (R)-5,6-Dihydrothymine
C21029  (R)-3-Ureidoisobutyrate
C21031  DNA 5-hydroxymethylcytosine

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