EC 4.1.1.22 HDC 1 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 4.1.3.4 HMGCL 1 reactions
HMG-CoA lyase deficiency: hypoketotic hypoglycaemia with metabolic acidosis
EC 1.1.1.34 HMGCR 1 reactions
Target of statins; SREBP-2 regulated
EC 1.13.11.5 HGD 1 reactions
Alkaptonuria: dark urine on standing, ochronosis, arthritis
EC 3.1.1.79 LIPE 1 reactions
Activated by PKA (glucagon/epinephrine), inhibited by insulin
1 reactions
EC 1.1.1.205 IMPDH1 / IMPDH2 1 reactions
EC 1.1.1.41 IDH3 1 reactions
Rate-limiting TCA step; IDH1/2 neomorphic mutations produce 2-hydroxyglutarate in glioma/AML
EC 5.3.3.2 IDI1 1 reactions
1 reactions
EC 1.1.1.35 HADH / HADHA 1 reactions
MCAD deficiency: hypoketotic hypoglycaemia, dicarboxylic aciduria, SIDS-like presentation
EC 1.1.1.27 LDHA/LDHB 1 reactions
Regenerates NAD+ anaerobically; LDHA deficiency causes exercise-induced myoglobinuria
EC 1.1.1.27 LDHA/LDHB 1 reactions
Regenerates NAD+ anaerobically; LDHA deficiency causes exercise-induced myoglobinuria
EC 5.4.99.7 LSS 1 reactions
EC 2.3.1.43 LCAT 1 reactions
LCAT deficiency: corneal opacity, anaemia, proteinuria; low HDL
1 reactions
EC 3.1.1.34 LPL 1 reactions
LPL or apoC-II deficiency: familial chylomicronaemia (type I), pancreatitis
1 reactions
1 reactions
EC 3.3.2.6 / 4.4.1.20 LTA4H / LTC4S 1 reactions