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

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Propionyl-CoA carboxylase (PCC) RL

EC 6.4.1.3 PCCA/PCCB 1 reactions

Propionic acidaemia; biotin-dependent

Ribonucleotide reductase (RNR, class Ia) RL

EC 1.17.4.1 RRM1/RRM2 1 reactions

Target of hydroxyurea; allosterically controlled by dNTPs

Serine palmitoyltransferase (SPT) RL

EC 2.3.1.50 SPTLC1/2 1 reactions

Rate-limiting for sphingolipid synthesis; SPTLC1 mutations cause HSAN type I

Succinyl-CoA:3-ketoacid CoA transferase (SCOT / OXCT1) RL

EC 2.8.3.5 OXCT1 1 reactions

SCOT deficiency: ketoacidosis; absent in liver, which is why liver cannot use ketones

Tryptophan hydroxylase (TPH1/2) RL

EC 1.14.16.4 TPH1 / TPH2 1 reactions

Tyrosine hydroxylase (TH) RL

EC 1.14.16.2 TH 1 reactions

Rate-limiting for catecholamines; BH4-dependent

UDP-glucose dehydrogenase (UGDH) RL

EC 1.1.1.22 UGDH 1 reactions

Supplies UDP-glucuronate for conjugation and GAG synthesis

Vitamin K epoxide reductase (VKORC1) RL

EC 1.17.4.4 VKORC1 1 reactions

Warfarin target; VKORC1 genotype drives dose variability