KEGG   PATHWAY: ko00240Help
Entry
ko00240                     Pathway                                

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

All organismsOrtholog 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
D00488  
Pyrimethamine (JAN/USP/INN)
D02487  
Proguanil hydrochloride (JAN)
D03034  
Azelaic acid (USAN/INN)
D03771  
Diaveridine (USAN/INN)
D08428  
Proguanil (INN)
Other DBs
BSID: 
GO: 
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]
K00945  
cytidylate kinase [EC:2.7.4.14]
K13799  
pantoate ligase / cytidylate kinase [EC:2.7.4.14 6.3.2.1]
K13800  
UMP-CMP kinase [EC:2.7.4.14 2.7.4.-]
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]
K01519  
inosine triphosphate pyrophosphatase [EC:3.6.1.19]
K01937  
CTP synthase [EC:6.3.4.2]
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 RPC10
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 I [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]
K01081  
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]
K08722  
5'-nucleotidase [EC:3.1.3.5]
K08723  
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]
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  
malonic semialdehyde reductase [EC:1.1.1.-]
K09019  
3-hydroxypropanoate dehydrogenase [EC:1.1.1.-]
K00756  
pyrimidine-nucleoside phosphorylase [EC:2.4.2.2]
K10213  
ribosylpyrimidine nucleosidase [EC:3.2.2.8]
K01485  
cytosine deaminase [EC:3.5.4.1]
K04765  
ATP diphosphatase [EC:3.6.1.8]
K00384  
thioredoxin reductase (NADPH) [EC:1.8.1.9]
K00527  
ribonucleoside-triphosphate reductase [EC:1.17.4.2]
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]
K01493  
dCMP deaminase [EC:3.5.4.12]
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  
pseudouridine-5'-phosphate glycosidase [EC:3.2.-.-]
K16330  
pseudouridine-5'-phosphate glycosidase / pseudouridine kinase [EC:2.7.1.83 3.2.-.-]
K01718  
pseudouridylate synthase [EC: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
C00342  
Thioredoxin
C00343  
Thioredoxin disulfide
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
C00906  
5,6-Dihydrothymine
C01013  
3-Hydroxypropanoate
C01103  
Orotidine 5'-phosphate
C01168  
Pseudouridine 5'-phosphate
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
C05100  
3-Ureidoisobutyrate
C05145  
3-Aminoisobutyric acid
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

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