KEGG PATHWAY: hsa00240

PATHWAY: hsa00240

Entry

hsa00240 Pathway

Name

Pyrimidine metabolism - Homo sapiens (human)

Class

Metabolism; Nucleotide metabolism

Pathway map

hsa00240

Pyrimidine metabolism

Module

hsa_M00046	Pyrimidine degradation, uracil => beta-alanine, thymine => 3-aminoisobutanoate [PATH:hsa00240]
hsa_M00051	Uridine monophosphate biosynthesis, glutamine (+ PRPP) => UMP [PATH:hsa00240]
hsa_M00052	Pyrimidine ribonucleotide biosynthesis, UMP => UDP/UTP,CDP/CTP [PATH:hsa00240]
hsa_M00053	Pyrimidine deoxyribonuleotide biosynthesis, CDP/CTP => dCDP/dCTP,dTDP/dTTP [PATH:hsa00240]

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

D00341	Hydroxycarbamide (JAN/INN)
D00584	Fluorouracil (JP16/USP/INN)
D00749	Leflunomide (JAN/USAN/INN)
D01064	Raltitrexed (JAN/USAN/INN)
D01155	Gemcitabine hydrochloride (JAN/USAN)
D01223	Capecitabine (JAN/USAN/INN)
D01244	Tegafur (JP16/USAN/INN)
D01309	Doxifluridine (JP16/INN)
D01784	Carmofur (JP16/INN)
D01846	Gimeracil (JAN/INN)
D02368	Gemcitabine (USAN/INN)
D03154	Brequinar sodium (USAN)
D03378	Caracemide (USAN)
D03546	Clofarabine (JAN/USAN/INN)
D03828	Pemetrexed disodium (USAN)
D04197	Floxuridine (USP/INN)
D04245	Forodesine hydrochloride (JAN/USAN)
D04964	Metesind glucuronate (USAN)
D06503	Pemetrexed sodium hydrate (JAN)
D06596	Forodesine (USAN/INN)
D07472	Pemetrexed (INN)
D07974	Fluorouracil sodium salt
D10172	Teriflunomide (USAN)
D10222	Gemcitabine elaidate (USAN/INN)

Other DBs

BSID:

82946

GO:

0006220

Organism

Homo sapiens (human) [GN:hsa]

Gene

790	CAD; carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase [KO:K11540] [EC:3.5.2.3 2.1.3.2 6.3.5.5]
1723	DHODH; dihydroorotate dehydrogenase (quinone) [KO:K00254] [EC:1.3.5.2]
7372	UMPS; uridine monophosphate synthetase [KO:K13421] [EC:4.1.1.23 2.4.2.10]
51727	CMPK1; cytidine monophosphate (UMP-CMP) kinase 1, cytosolic [KO:K13800] [EC:2.7.4.14 2.7.4.-]
129607	CMPK2; cytidine monophosphate (UMP-CMP) kinase 2, mitochondrial [KO:K13809] [EC:2.7.4.14]
50808	AK3; adenylate kinase 3 [KO:K00944] [EC:2.7.4.10]
10201	NME6; NME/NM23 nucleoside diphosphate kinase 6 [KO:K00940] [EC:2.7.4.6]
29922	NME7; NME/NM23 family member 7 [KO:K00940] [EC:2.7.4.6]
4831	NME2; NME/NM23 nucleoside diphosphate kinase 2 [KO:K00940] [EC:2.7.4.6]
4833	NME4; NME/NM23 nucleoside diphosphate kinase 4 [KO:K00940] [EC:2.7.4.6]
4830	NME1; NME/NM23 nucleoside diphosphate kinase 1 [KO:K00940] [EC:2.7.4.6]
8382	NME5; NME/NM23 family member 5 [KO:K00940] [EC:2.7.4.6]
4832	NME3; NME/NM23 nucleoside diphosphate kinase 3 [KO:K00940] [EC:2.7.4.6]
654364	NME1-NME2; NME1-NME2 readthrough [KO:K00940] [EC:2.7.4.6]
87178	PNPT1; polyribonucleotide nucleotidyltransferase 1 [KO:K00962] [EC:2.7.7.8]
956	ENTPD3; ectonucleoside triphosphate diphosphohydrolase 3 [KO:K01510] [EC:3.6.1.5]
377841	ENTPD8; ectonucleoside triphosphate diphosphohydrolase 8 [KO:K01510] [EC:3.6.1.5]
953	ENTPD1; ectonucleoside triphosphate diphosphohydrolase 1 [KO:K01510] [EC:3.6.1.5]
124583	CANT1; calcium activated nucleotidase 1 [KO:K12304] [EC:3.6.1.6]
9583	ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
957	ENTPD5; ectonucleoside triphosphate diphosphohydrolase 5 [KO:K01511] [EC:3.6.1.6]
955	ENTPD6; ectonucleoside triphosphate diphosphohydrolase 6 (putative) [KO:K01511] [EC:3.6.1.6]
3704	ITPA; inosine triphosphatase (nucleoside triphosphate pyrophosphatase) [KO:K01519] [EC:3.6.1.19]
1503	CTPS1; CTP synthase 1 [KO:K01937] [EC:6.3.4.2]
56474	CTPS2; CTP synthase 2 [KO:K01937] [EC:6.3.4.2]
25885	POLR1A; polymerase (RNA) I polypeptide A, 194kDa [KO:K02999] [EC:2.7.7.6]
84172	POLR1B; polymerase (RNA) I polypeptide B, 128kDa [KO:K03002] [EC:2.7.7.6]
30834	ZNRD1; zinc ribbon domain containing 1 [KO:K03000]
221830	TWISTNB; TWIST neighbor [KO:K03004]
64425	POLR1E; polymerase (RNA) I polypeptide E, 53kDa [KO:K03005]
5430	POLR2A; polymerase (RNA) II (DNA directed) polypeptide A, 220kDa [KO:K03006] [EC:2.7.7.6]
5431	POLR2B; polymerase (RNA) II (DNA directed) polypeptide B, 140kDa [KO:K03010] [EC:2.7.7.6]
5432	POLR2C; polymerase (RNA) II (DNA directed) polypeptide C, 33kDa [KO:K03011]
5433	POLR2D; polymerase (RNA) II (DNA directed) polypeptide D [KO:K03012]
5434	POLR2E; polymerase (RNA) II (DNA directed) polypeptide E, 25kDa [KO:K03013]
5435	POLR2F; polymerase (RNA) II (DNA directed) polypeptide F [KO:K03014]
5436	POLR2G; polymerase (RNA) II (DNA directed) polypeptide G [KO:K03015]
5437	POLR2H; polymerase (RNA) II (DNA directed) polypeptide H [KO:K03016]
5438	POLR2I; polymerase (RNA) II (DNA directed) polypeptide I, 14.5kDa [KO:K03017]
5441	POLR2L; polymerase (RNA) II (DNA directed) polypeptide L, 7.6kDa [KO:K03007]
5439	POLR2J; polymerase (RNA) II (DNA directed) polypeptide J, 13.3kDa [KO:K03008]
548644	POLR2J3; polymerase (RNA) II (DNA directed) polypeptide J3 [KO:K03008]
246721	POLR2J2; polymerase (RNA) II (DNA directed) polypeptide J2 [KO:K03008]
5440	POLR2K; polymerase (RNA) II (DNA directed) polypeptide K, 7.0kDa [KO:K03009]
11128	POLR3A; polymerase (RNA) III (DNA directed) polypeptide A, 155kDa [KO:K03018] [EC:2.7.7.6]
55703	POLR3B; polymerase (RNA) III (DNA directed) polypeptide B [KO:K03021] [EC:2.7.7.6]
10623	POLR3C; polymerase (RNA) III (DNA directed) polypeptide C (62kD) [KO:K03023]
101060460	DNA-directed RNA polymerase III subunit RPC3-like; K03023 DNA-directed RNA polymerase III subunit RPC3 [KO:K03023]
661	POLR3D; polymerase (RNA) III (DNA directed) polypeptide D, 44kDa [KO:K03026]
55718	POLR3E; polymerase (RNA) III (DNA directed) polypeptide E (80kD) [KO:K14721]
101060521	DNA-directed RNA polymerase III subunit RPC5-like; K14721 DNA-directed RNA polymerase III subunit RPC5 [KO:K14721]
9533	POLR1C; polymerase (RNA) I polypeptide C, 30kDa [KO:K03027]
51728	POLR3K; polymerase (RNA) III (DNA directed) polypeptide K, 12.3 kDa [KO:K03019]
51082	POLR1D; polymerase (RNA) I polypeptide D, 16kDa [KO:K03020]
171568	POLR3H; polymerase (RNA) III (DNA directed) polypeptide H (22.9kD) [KO:K03022]
84265	POLR3GL; polymerase (RNA) III (DNA directed) polypeptide G (32kD)-like [KO:K03024]
10622	POLR3G; polymerase (RNA) III (DNA directed) polypeptide G (32kD) [KO:K03024]
101060555	DNA-directed RNA polymerase III subunit RPC7-like; K03024 DNA-directed RNA polymerase III subunit RPC7 [KO:K03024]
10621	POLR3F; polymerase (RNA) III (DNA directed) polypeptide F, 39 kDa [KO:K03025]
5422	POLA1; polymerase (DNA directed), alpha 1, catalytic subunit [KO:K02320] [EC:2.7.7.7]
23649	POLA2; polymerase (DNA directed), alpha 2, accessory subunit [KO:K02321]
5557	PRIM1; primase, DNA, polypeptide 1 (49kDa) [KO:K02684] [EC:2.7.7.-]
5558	PRIM2; primase, DNA, polypeptide 2 (58kDa) [KO:K02685] [EC:2.7.7.-]
5424	POLD1; polymerase (DNA directed), delta 1, catalytic subunit [KO:K02327] [EC:2.7.7.7]
5425	POLD2; polymerase (DNA directed), delta 2, accessory subunit [KO:K02328]
10714	POLD3; polymerase (DNA-directed), delta 3, accessory subunit [KO:K03504]
57804	POLD4; polymerase (DNA-directed), delta 4, accessory subunit [KO:K03505]
5426	POLE; polymerase (DNA directed), epsilon, catalytic subunit [KO:K02324] [EC:2.7.7.7]
5427	POLE2; polymerase (DNA directed), epsilon 2, accessory subunit [KO:K02325] [EC:2.7.7.7]
54107	POLE3; polymerase (DNA directed), epsilon 3, accessory subunit [KO:K02326] [EC:2.7.7.7]
56655	POLE4; polymerase (DNA-directed), epsilon 4, accessory subunit [KO:K03506] [EC:2.7.7.7]
83549	UCK1; uridine-cytidine kinase 1 [KO:K00876] [EC:2.7.1.48]
7371	UCK2; uridine-cytidine kinase 2 [KO:K00876] [EC:2.7.1.48]
54963	UCKL1; uridine-cytidine kinase 1-like 1 [KO:K00876] [EC:2.7.1.48]
22978	NT5C2; 5'-nucleotidase, cytosolic II [KO:K01081] [EC:3.1.3.5]
84618	NT5C1A; 5'-nucleotidase, cytosolic IA [KO:K01081] [EC:3.1.3.5]
4907	NT5E; 5'-nucleotidase, ecto (CD73) [KO:K01081] [EC:3.1.3.5]
93034	NT5C1B; 5'-nucleotidase, cytosolic IB [KO:K01081] [EC:3.1.3.5]
30833	NT5C; 5', 3'-nucleotidase, cytosolic [KO:K01081] [EC:3.1.3.5]
56953	NT5M; 5',3'-nucleotidase, mitochondrial [KO:K01081] [EC:3.1.3.5]
51251	NT5C3A; 5'-nucleotidase, cytosolic IIIA [KO:K01081] [EC:3.1.3.5]
115024	NT5C3B; 5'-nucleotidase, cytosolic IIIB [KO:K01081] [EC:3.1.3.5]
100526794	NT5C1B-RDH14; NT5C1B-RDH14 readthrough [KO:K01081] [EC:3.1.3.5]
151531	UPP2; uridine phosphorylase 2 [KO:K00757] [EC:2.4.2.3]
7378	UPP1; uridine phosphorylase 1 [KO:K00757] [EC:2.4.2.3]
139596	UPRT; uracil phosphoribosyltransferase (FUR1) homolog (S. cerevisiae) [KO:K00761] [EC:2.4.2.9]
1806	DPYD; dihydropyrimidine dehydrogenase [KO:K00207] [EC:1.3.1.2]
1807	DPYS; dihydropyrimidinase [KO:K01464] [EC:3.5.2.2]
51733	UPB1; ureidopropionase, beta [KO:K01431] [EC:3.5.1.6]
7296	TXNRD1; thioredoxin reductase 1 [KO:K00384] [EC:1.8.1.9]
10587	TXNRD2; thioredoxin reductase 2 [KO:K00384] [EC:1.8.1.9]
114112	TXNRD3; thioredoxin reductase 3 [KO:K00384] [EC:1.8.1.9]
6240	RRM1; ribonucleotide reductase M1 [KO:K10807] [EC:1.17.4.1]
50484	RRM2B; ribonucleotide reductase M2 B (TP53 inducible) [KO:K10808] [EC:1.17.4.1]
6241	RRM2; ribonucleotide reductase M2 [KO:K10808] [EC:1.17.4.1]
79077	DCTPP1; dCTP pyrophosphatase 1 [KO:K16904] [EC:3.6.1.12]
1854	DUT; deoxyuridine triphosphatase [KO:K01520] [EC:3.6.1.23]
7298	TYMS; thymidylate synthetase [KO:K00560] [EC:2.1.1.45]
978	CDA; cytidine deaminase [KO:K01489] [EC:3.5.4.5]
1890	TYMP; thymidine phosphorylase [KO:K00758] [EC:2.4.2.4]
4860	PNP; purine nucleoside phosphorylase [KO:K03783] [EC:2.4.2.1]
1633	DCK; deoxycytidine kinase [KO:K00893] [EC:2.7.1.74]
1635	DCTD; dCMP deaminase [KO:K01493] [EC:3.5.4.12]
7084	TK2; thymidine kinase 2, mitochondrial [KO:K00857] [EC:2.7.1.21]
7083	TK1; thymidine kinase 1, soluble [KO:K00857] [EC:2.7.1.21]
1841	DTYMK; deoxythymidylate kinase (thymidylate kinase) [KO:K00943] [EC:2.7.4.9]
318	NUDT2; nudix (nucleoside diphosphate linked moiety X)-type motif 2 [KO:K01518] [EC:3.6.1.17]

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

KO pathway

ko00240

All links

Ontology (1)   
   KEGG BRITE (1)   
Pathway (4)   
   KEGG MODULE (3)   
   BioSystems (1)   
Disease (9)   
   KEGG DISEASE (9)   
Drug (24)   
   KEGG DRUG (24)   
Chemical substance (1)   
   KEGG COMPOUND (1)   
Genome (1)   
   KEGG GENOME (1)   
Gene (107)   
   KEGG GENES (107)   
All databases (147)   

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