EC 3.5.1.6 UPB1 2 reactions
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.
EC 2.6.1.42 BCAT1 / BCAT2 2 reactions
EC 1.14.14.23 CYP7A1 2 reactions
Rate-limiting for bile acid synthesis; up-regulated by bile acid sequestrants
EC 2.3.3.1 CS 2 reactions
Entry step of TCA; inhibited by citrate, ATP, NADH, succinyl-CoA
EC 1.1.1.8 GPD1 2 reactions
EC 1.1.1.37 MDH1 2 reactions
EC 1.1.1.30 BDH1 2 reactions
EC 3.5.4.12 DCTD 2 reactions
EC 2.7.1.74 / 2.7.1.113 DCK / DGUOK 2 reactions
EC 3.5.2.2 DPYS 2 reactions
EC 1.3.1.2 DPYD 2 reactions
DPD deficiency: severe 5-fluorouracil toxicity
EC 2.3.1.85 FASN 2 reactions
EC 4.1.2.13 ALDOA / ALDOB / ALDOC 2 reactions
EC 2.1.2.2 GART 2 reactions
EC 3.1.3.9 G6PC / SLC37A4 2 reactions
von Gierke disease (GSD I): fasting hypoglycaemia, lactic acidosis, hyperuricaemia
EC 2.5.1.18 GST 2 reactions
Conjugates NAPQI; depletion underlies paracetamol hepatotoxicity
EC 2.7.1.1 / 2.7.1.2 HK1-3 / GCK 2 reactions
GCK mutations: MODY2; HK1 deficiency: haemolytic anaemia
EC 2.7.1.1 / 2.7.1.2 HK1-3 / GCK 2 reactions
GCK mutations: MODY2; HK1 deficiency: haemolytic anaemia
EC 2.4.2.8 HPRT1 2 reactions
Lesch-Nyhan syndrome: hyperuricaemia, self-mutilation, choreoathetosis, intellectual disability
EC 3.1.1.34 LPL 2 reactions
LPL or apoC-II deficiency: familial chylomicronaemia (type I), pancreatitis