Enzymes & cofactors

Search by name, gene symbol, EC number or the disorder it causes. Open any enzyme to see every reaction it catalyses, what accelerates it and what inhibits it.

Enzymes Cofactor terms Vitamins & minerals

Cofactor / coenzyme

Restrict to

Reset

Dihydrolipoyl dehydrogenase (E3) RL

EC 1.8.1.4 DLD 4 reactions

Dihydrolipoyl transacetylase (E2) RL

EC 2.3.1.12 DLAT 3 reactions

Thymidylate synthase (TYMS) RL

EC 2.1.1.45 TYMS 3 reactions

Target of 5-fluorouracil (via FdUMP)

Aldose reductase (AKR1B1) RL

EC 1.1.1.21 AKR1B1 2 reactions

Target of aldose reductase inhibitors; polyol accumulation in diabetes

Cholesterol 7-alpha-hydroxylase (CYP7A1) RL

EC 1.14.14.23 CYP7A1 2 reactions

Rate-limiting for bile acid synthesis; up-regulated by bile acid sequestrants

Dihydropyrimidine dehydrogenase (DPD) RL

EC 1.3.1.2 DPYD 2 reactions

DPD deficiency: severe 5-fluorouracil toxicity

GAR transformylase (GART) RL

EC 2.1.2.2 GART 2 reactions

Phosphofructokinase-1 (PFK-1) RL

EC 2.7.1.11 PFKM/PFKL 2 reactions

Tarui disease (GSD VII): exercise intolerance, haemolysis

1-alpha-hydroxylase (CYP27B1) RL

EC 1.14.15.18 CYP27B1 1 reactions

Vitamin D-dependent rickets type 1; stimulated by PTH, suppressed by FGF-23

Acetyl-CoA carboxylase (ACC1) RL

EC 6.4.1.2 ACACA 1 reactions

Biotin-dependent; inhibited by AMPK; ACC inhibitors studied in NASH/obesity

Adenylosuccinate synthetase RL

EC 6.3.4.4 ADSS2 1 reactions

Aldolase B (fructose-1-phosphate aldolase) RL

EC 4.1.2.13 ALDOB 1 reactions

Hereditary fructose intolerance: hypoglycaemia, vomiting after fructose/sucrose

Bilirubin UDP-glucuronosyltransferase (UGT1A1) RL

EC 2.4.1.17 UGT1A1 1 reactions

Gilbert syndrome (mild), Crigler-Najjar types I and II

Branched-chain alpha-ketoacid dehydrogenase complex (BCKDH) RL

EC 1.2.4.4 BCKDHA/B, DBT 1 reactions

Maple syrup urine disease: burnt-sugar urine, ketoacidosis; thiamine-responsive variants

Carbamoyl phosphate synthetase I (CPS I) RL

EC 6.3.4.16 CPS1 1 reactions

CPS I deficiency: hyperammonaemia, normal orotic acid

Carnitine palmitoyltransferase I (CPT-1) RL

EC 2.3.1.21 CPT1A 1 reactions

CPT-I deficiency: hypoketotic hypoglycaemia, hepatomegaly

CPS-II domain of carbamoyl-phosphate synthetase II (CPS-II) RL

EC 6.3.5.5 CAD 1 reactions

CTP synthetase RL

EC 6.3.4.2 CTPS1 1 reactions

CTP:phosphocholine cytidylyltransferase (CCT) RL

EC 2.7.7.15 PCYT1A 1 reactions

Cyclooxygenase (COX-1 / COX-2; PTGS1/2) RL

EC 1.14.99.1 PTGS1/PTGS2 1 reactions

Irreversibly acetylated by aspirin; NSAID and coxib target