KEGG   PATHWAY: hsa00240Help
Entry
hsa00240                    Pathway                                

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

Ortholog table
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]
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)
D10596  Pemetrexed sodium hemipentahydrate (JAN)
D10803  Plevitrexed (INN)
D10963  Oleclumab (USAN)
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/uridine monophosphate kinase 1 [KO:K13800] [EC:2.7.4.14]
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]
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]
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; RNA polymerase I subunit A [KO:K02999] [EC:2.7.7.6]
84172  POLR1B; RNA polymerase 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; RNA polymerase I subunit E [KO:K03005]
5430  POLR2A; RNA polymerase II subunit A [KO:K03006] [EC:2.7.7.6]
5431  POLR2B; RNA polymerase II subunit B [KO:K03010] [EC:2.7.7.6]
5432  POLR2C; RNA polymerase II subunit C [KO:K03011]
5433  POLR2D; RNA polymerase II subunit D [KO:K03012]
5434  POLR2E; RNA polymerase II subunit E [KO:K03013]
5435  POLR2F; RNA polymerase II subunit F [KO:K03014]
5436  POLR2G; RNA polymerase II subunit G [KO:K03015]
5437  POLR2H; RNA polymerase II subunit H [KO:K03016]
5438  POLR2I; RNA polymerase II subunit I [KO:K03017]
5441  POLR2L; RNA polymerase II subunit L [KO:K03007]
5439  POLR2J; RNA polymerase II subunit J [KO:K03008]
548644  POLR2J3; RNA polymerase II subunit J3 [KO:K03008]
246721  POLR2J2; RNA polymerase II subunit J2 [KO:K03008]
5440  POLR2K; RNA polymerase II subunit K [KO:K03009]
11128  POLR3A; RNA polymerase III subunit A [KO:K03018] [EC:2.7.7.6]
55703  POLR3B; RNA polymerase III subunit B [KO:K03021] [EC:2.7.7.6]
10623  POLR3C; RNA polymerase III subunit C [KO:K03023]
661  POLR3D; RNA polymerase III subunit D [KO:K03026]
55718  POLR3E; RNA polymerase III subunit E [KO:K14721]
101060521  DNA-directed RNA polymerase III subunit RPC5 [KO:K14721]
9533  POLR1C; RNA polymerase I and III subunit C [KO:K03027]
51728  POLR3K; RNA polymerase III subunit K [KO:K03019]
51082  POLR1D; RNA polymerase I and III subunit D [KO:K03020]
171568  POLR3H; RNA polymerase III subunit H [KO:K03022]
84265  POLR3GL; RNA polymerase III subunit G like [KO:K03024]
10622  POLR3G; RNA polymerase III subunit G [KO:K03024]
10621  POLR3F; RNA polymerase III subunit F [KO:K03025]
5422  POLA1; DNA polymerase alpha 1, catalytic subunit [KO:K02320] [EC:2.7.7.7]
23649  POLA2; DNA polymerase alpha 2, accessory subunit [KO:K02321]
5557  PRIM1; DNA primase subunit 1 [KO:K02684] [EC:2.7.7.-]
5558  PRIM2; DNA primase subunit 2 [KO:K02685] [EC:2.7.7.-]
5424  POLD1; DNA polymerase delta 1, catalytic subunit [KO:K02327] [EC:2.7.7.7]
5425  POLD2; DNA polymerase delta 2, accessory subunit [KO:K02328]
10714  POLD3; DNA polymerase delta 3, accessory subunit [KO:K03504]
57804  POLD4; DNA polymerase delta 4, accessory subunit [KO:K03505]
5426  POLE; DNA polymerase epsilon, catalytic subunit [KO:K02324] [EC:2.7.7.7]
5427  POLE2; DNA polymerase epsilon 2, accessory subunit [KO:K02325] [EC:2.7.7.7]
54107  POLE3; DNA polymerase epsilon 3, accessory subunit [KO:K02326] [EC:2.7.7.7]
56655  POLE4; DNA polymerase 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]
100526794  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]
5167  ENPP1; ectonucleotide pyrophosphatase/phosphodiesterase 1 [KO:K01513] [EC:3.6.1.9 3.1.4.1]
5169  ENPP3; ectonucleotide pyrophosphatase/phosphodiesterase 3 [KO:K01513] [EC:3.6.1.9 3.1.4.1]
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
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
ko00240   

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