Page last updated: 2024-10-06

Pyrimidine metabolism and related diseases

Proteins (18)

ProteinSynonymsTaxonomy
Glutaminase liver isoform, mitochondrialGLS; EC 3.5.1.2; L-glutaminase; L-glutamine amidohydrolaseHomo sapiens (human)
Thymidine kinase 2, mitochondrialEC 2.7.1.21; Mt-TKHomo sapiens (human)
Thymidylate synthaseTS; TSase; EC 2.1.1.45Homo sapiens (human)
4-aminobutyrate aminotransferase, mitochondrialEC 2.6.1.19; (S)-3-amino-2-methylpropionate transaminase; 2.6.1.22; GABA aminotransferase; GABA-AT; Gamma-amino-N-butyrate transaminase; GABA transaminase; GABA-T; L-AIBATHomo sapiens (human)
Dihydropyrimidine dehydrogenase [NADP(+)]DHPDHase; DPD; EC 1.3.1.2; Dihydrothymine dehydrogenase; Dihydrouracil dehydrogenaseHomo sapiens (human)
Thymidine phosphorylaseTP; EC 2.4.2.4; Gliostatin; Platelet-derived endothelial cell growth factor; PD-ECGF; TdRPaseHomo sapiens (human)
Ribonucleoside-diphosphate reductase large subunitEC 1.17.4.1; Ribonucleoside-diphosphate reductase subunit M1; Ribonucleotide reductase large subunitHomo sapiens (human)
Ribonucleoside-diphosphate reductase subunit M2EC 1.17.4.1; Ribonucleotide reductase small chain; Ribonucleotide reductase small subunitHomo sapiens (human)
Beta-ureidopropionaseEC 3.5.1.6; BUP-1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolaseHomo sapiens (human)
Carbamoyl-phosphate synthase [ammonia], mitochondrialEC 6.3.4.16; Carbamoyl-phosphate synthetase I; CPSase IHomo sapiens (human)
Dihydroorotate dehydrogenase (quinone), mitochondrialDHOdehase; EC 1.3.5.2; Dihydroorotate oxidaseHomo sapiens (human)
Ornithine transcarbamylase, mitochondrialOTCase; EC 2.1.3.3; Ornithine carbamoyltransferase, mitochondrialHomo sapiens (human)
DihydropyrimidinaseDHP; DHPase; EC 3.5.2.2; Dihydropyrimidine amidohydrolase; HydantoinaseHomo sapiens (human)
Ribonucleoside-diphosphate reductase subunit M2 BEC 1.17.4.1; TP53-inducible ribonucleotide reductase M2 B; p53-inducible ribonucleotide reductase small subunit 2-like protein; p53R2Homo sapiens (human)
5'(3')-deoxyribonucleotidase, cytosolic typeEC 3.1.3.-; Cytosolic 5',3'-pyrimidine nucleotidase; Deoxy-5'-nucleotidase 1; dNT-1Homo sapiens (human)
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrialMMSDH; Malonate-semialdehyde dehydrogenase [acylating]; EC 1.2.1.18; EC 1.2.1.27; Aldehyde dehydrogenase family 6 member A1Homo sapiens (human)
Alanine--glyoxylate aminotransferase 2, mitochondrialAGT 2; EC 2.6.1.44; (R)-3-amino-2-methylpropionate--pyruvate transaminase; 2.6.1.40; Beta-ALAAT II; Beta-alanine-pyruvate aminotransferase; D-AIBATHomo sapiens (human)
Cytosolic 5'-nucleotidase 3AEC 3.1.3.5; 7-methylguanosine phosphate-specific 5'-nucleotidase; 7-methylguanosine nucleotidase; 3.1.3.91; Cytosolic 5'-nucleotidase 3; Cytosolic 5'-nucleotidase III; cN-III; Pyrimidine 5'-nucleotidase 1; P5'N-1; P5N-1; PN-I; Uridine 5'-monophosphate hydHomo sapiens (human)

Compounds (30)

CompoundDescription
uridine diphosphateA uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety.
Ammonium
coenzyme q10Ubiquinone ring with a chain of 10 isoprene units; redox equilibrium with ubiqunol serving in mitochondrial inner membrane to transfer electrons; presence during reconstitution of acetylcholine receptor into phospholipid vesicles yields vesicles active in catalyzing carbamylcholine-sensitive Na+ flux; coenzyme Q10 depletion has been noted with use of statins
Aspartate
uridineA ribonucleoside in which RIBOSE is linked to URACIL.
Uridine TriphosphateUridine 5'-(tetrahydrogen triphosphate). A uracil nucleotide containing three phosphate groups esterified to the sugar moiety.
phosphoribosyl pyrophosphateThe key substance in the biosynthesis of histidine, tryptophan, and purine and pyrimidine nucleotides.
hydrogen carbonate
ubiquinolreduced forms of ubiquinone; see also record for ubiquinol 10
thymineOne of four constituent bases of DNA.
uracilOne of four nucleotide bases in the nucleic acid RNA.
deoxyuridine2'-Deoxyuridine. An antimetabolite that is converted to deoxyuridine triphosphate during DNA synthesis. Laboratory suppression of deoxyuridine is used to diagnose megaloblastic anemias due to vitamin B12 and folate deficiencies.
Thymidine Monophosphate5-Thymidylic acid. A thymine nucleotide containing one phosphate group esterified to the deoxyribose moiety.
Carbamyl PhosphateThe monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).
glutamate
thymidineA nucleoside in which THYMINE is linked to DEOXYRIBOSE.
cytidineA pyrimidine nucleoside that is composed of the base CYTOSINE linked to the five-carbon sugar D-RIBOSE.
dihydrouracil
polyglutamineRN given for (D)-isomer
Cytidine MonophosphateCytidine (dihydrogen phosphate). A cytosine nucleotide containing one phosphate group esterified to the sugar moiety in the 2', 3' or 5' position.
Cytidine TriphosphateCytidine 5'-(tetrahydrogen triphosphate). A cytosine nucleotide containing three phosphate groups esterified to the sugar moiety.
Uridine Monophosphate5'-Uridylic acid. A uracil nucleotide containing one phosphate group esterified to the sugar moiety in the 2', 3' or 5' position.
2'-deoxyuridylic acidRN given refers to parent cpd
Cytidine DiphosphateCytidine 5'-(trihydrogen diphosphate). A cytosine nucleotide containing two phosphate groups esterified to the sugar moiety. Synonyms: CRPP; cytidine pyrophosphate.
deoxyuridine triphosphate
acetyl coenzyme aAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
propionyl-coenzyme ARN given refers to parent cpd
orotidylic acidstructure
orotidineRN given refers to parent cpd; structure
5,6-dihydrothymineRN given refers to parent cpd