KEGG   PATHWAY: hsa00240Help
Entry
hsa00240                    Pathway                                

Name
Pyrimidine metabolism - Homo sapiens (human)
Class
Metabolism; Nucleotide metabolism
BRITE hierarchy
Pathway map
Pyrimidine metabolism
hsa00240

All organismsOrtholog table
Module
Pyrimidine degradation, uracil => beta-alanine, thymine => 3-aminoisobutanoate [PATH:hsa00240]
Uridine monophosphate biosynthesis, glutamine (+ PRPP) => UMP [PATH:hsa00240]
Pyrimidine ribonucleotide biosynthesis, UMP => UDP/UTP,CDP/CTP [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)
H01376  
Acrofacial dysostosis
H01390  
Mitochondrial neurogastrointestinal encephalomyopathy
Drug
D00341  
Hydroxyurea (USP)
D00584  
Fluorouracil (JP17/USP/INN)
D00749  
Leflunomide (JAN/USAN/INN)
D01064  
Raltitrexed (JAN/USAN/INN)
D01155  
Gemcitabine hydrochloride (JAN/USAN)
D01223  
Capecitabine (JAN/USAN/INN)
D01244  
Tegafur (JP17/USAN/INN)
D01309  
Doxifluridine (JP17/INN)
D01784  
Carmofur (JP17/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)
D10431  
Ulodesine (USAN)
D10432  
Ulodesine succinate (USAN)
Other DBs
BSID: 
GO: 
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/uridine monophosphate kinase 1 [KO:K13800] [EC:2.7.4.14 2.7.4.-]
129607  
CMPK2; cytidine/uridine monophosphate kinase 2 [KO:K13809] [EC:2.7.4.14]
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]
221264  
AK9; adenylate kinase 9 [KO:K18533] [EC:2.7.4.6 2.7.4.3]
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 [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 subunit A [KO:K02999] [EC:2.7.7.6]
84172  
POLR1B; polymerase (RNA) I subunit B [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 subunit E [KO:K03005]
5430  
POLR2A; polymerase (RNA) II subunit A [KO:K03006] [EC:2.7.7.6]
5431  
POLR2B; polymerase (RNA) II subunit B [KO:K03010] [EC:2.7.7.6]
5432  
POLR2C; polymerase (RNA) II subunit C [KO:K03011]
5433  
POLR2D; polymerase (RNA) II subunit D [KO:K03012]
5434  
POLR2E; polymerase (RNA) II subunit E [KO:K03013]
5435  
POLR2F; polymerase (RNA) II subunit F [KO:K03014]
5436  
POLR2G; polymerase (RNA) II subunit G [KO:K03015]
5437  
POLR2H; polymerase (RNA) II subunit H [KO:K03016]
5438  
POLR2I; polymerase (RNA) II subunit I [KO:K03017]
5441  
POLR2L; polymerase (RNA) II subunit L [KO:K03007]
5439  
POLR2J; polymerase (RNA) II subunit J [KO:K03008]
548644  
POLR2J3; polymerase (RNA) II subunit J3 [KO:K03008]
246721  
POLR2J2; polymerase (RNA) II subunit J2 [KO:K03008]
5440  
POLR2K; polymerase (RNA) II subunit K [KO:K03009]
11128  
POLR3A; polymerase (RNA) III subunit A [KO:K03018] [EC:2.7.7.6]
55703  
POLR3B; polymerase (RNA) III subunit B [KO:K03021] [EC:2.7.7.6]
10623  
POLR3C; polymerase (RNA) III subunit C [KO:K03023]
661  
POLR3D; polymerase (RNA) III subunit D [KO:K03026]
55718  
POLR3E; polymerase (RNA) III subunit E [KO:K14721]
DNA-directed RNA polymerase III subunit RPC5; K14721 DNA-directed RNA polymerase III subunit RPC5 [KO:K14721]
9533  
POLR1C; polymerase (RNA) I subunit C [KO:K03027]
51728  
POLR3K; polymerase (RNA) III subunit K [KO:K03019]
51082  
POLR1D; polymerase (RNA) I subunit D [KO:K03020]
171568  
POLR3H; polymerase (RNA) III subunit H [KO:K03022]
84265  
POLR3GL; polymerase (RNA) III subunit G like [KO:K03024]
10622  
POLR3G; polymerase (RNA) III subunit G [KO:K03024]
10621  
POLR3F; polymerase (RNA) III subunit F [KO:K03025]
5422  
POLA1; polymerase (DNA) alpha 1, catalytic subunit [KO:K02320] [EC:2.7.7.7]
23649  
POLA2; polymerase (DNA) alpha 2, accessory subunit [KO:K02321]
5557  
PRIM1; primase (DNA) subunit 1 [KO:K02684] [EC:2.7.7.-]
5558  
PRIM2; primase (DNA) subunit 2 [KO:K02685] [EC:2.7.7.-]
5424  
POLD1; polymerase (DNA) delta 1, catalytic subunit [KO:K02327] [EC:2.7.7.7]
5425  
POLD2; polymerase (DNA) delta 2, accessory subunit [KO:K02328]
10714  
POLD3; polymerase (DNA) delta 3, accessory subunit [KO:K03504]
57804  
POLD4; polymerase (DNA) delta 4, accessory subunit [KO:K03505]
5426  
POLE; polymerase (DNA) epsilon, catalytic subunit [KO:K02324] [EC:2.7.7.7]
5427  
POLE2; polymerase (DNA) epsilon 2, accessory subunit [KO:K02325] [EC:2.7.7.7]
54107  
POLE3; polymerase (DNA) epsilon 3, accessory subunit [KO:K02326] [EC:2.7.7.7]
56655  
POLE4; polymerase (DNA) 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]
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]
NT5C1B-RDH14; NT5C1B-RDH14 readthrough [KO:K01081] [EC:3.1.3.5]
4907  
NT5E; 5'-nucleotidase ecto [KO:K19970] [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 homolog [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; beta-ureidopropionase 1 [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 catalytic subunit M1 [KO:K10807] [EC:1.17.4.1]
50484  
RRM2B; ribonucleotide reductase regulatory TP53 inducible subunit M2B [KO:K10808] [EC:1.17.4.1]
6241  
RRM2; ribonucleotide reductase regulatory subunit 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 [KO:K00857] [EC:2.7.1.21]
1841  
DTYMK; deoxythymidylate kinase [KO:K00943] [EC:2.7.4.9]
318  
NUDT2; nudix hydrolase 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
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
KO pathway
 

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