KEGG PATHWAY: ko00240

PATHWAY: ko00240

Entry

ko00240 Pathway

Name

Pyrimidine metabolism

Class

Metabolism; Nucleotide metabolism

Pathway map

ko00240

Pyrimidine metabolism

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)

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:

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]
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]
K00944	nucleoside-triphosphate--adenylate kinase [EC:2.7.4.10]
K00940	nucleoside-diphosphate kinase [EC:2.7.4.6]
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]
K02428	dITP/XTP 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]
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 [EC:3.1.4.16]
K08693	3'-nucleotidase [EC:3.1.3.6]
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

All links

Ontology (2)   
   KEGG BRITE (1)   
   GO (1)   
Pathway (9)   
   KEGG PATHWAY (4)   
   KEGG MODULE (4)   
   BioSystems (1)   
Disease (9)   
   KEGG DISEASE (9)   
Drug (5)   
   KEGG DRUG (5)   
Chemical substance (66)   
   KEGG COMPOUND (66)   
Chemical reaction (65)   
   KEGG ENZYME (65)   
Gene (165)   
   KEGG ORTHOLOGY (165)   
All databases (321)   

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