EC 7.1.1.9 MT-CO1-3 1 reactions
Inhibited by cyanide, carbon monoxide, azide, H2S
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.
Showing enzymes whose reactions use H2O.
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EC 6.3.5.5 CAD 1 reactions
EC 6.3.4.2 CTPS1 1 reactions
EC 4.2.1.22 CBS 1 reactions
Homocystinuria: marfanoid habitus, DOWNWARD lens dislocation, thrombosis; B6-responsive forms
EC 4.4.1.1 CTH 1 reactions
EC 3.5.4.5 CDA 1 reactions
EC 2.3.3.10 HMGCS1 1 reactions
EC 3.5.2.3 CAD 1 reactions
EC 4.2.1.17 ECHS1 1 reactions
1 reactions
EC 7.1.2.2 ATP5 genes 1 reactions
Inhibited by oligomycin; bypassed by uncouplers (DNP, UCP1)
EC 6.3.5.3 PFAS 1 reactions
EC 3.1.3.11 FBP1 1 reactions
FBPase deficiency: fasting hypoglycaemia, lactic acidosis, ketosis
EC 4.2.1.2 FH 1 reactions
FH mutations: hereditary leiomyomatosis and renal cell carcinoma
EC 3.7.1.2 FAH 1 reactions
Tyrosinaemia type I: liver failure, renal Fanconi, cabbage odour; treated with nitisinone
EC 1.4.1.3 GLUD1 1 reactions
Activating mutations: hyperinsulinism-hyperammonaemia syndrome
EC 3.5.1.2 GLS 1 reactions
EC 2.4.2.14 PPAT 1 reactions
Committed step of purine synthesis; feedback-inhibited by AMP/GMP; azaserine site
EC 3.2.1.33 AGL 1 reactions
Cori-Forbes disease (GSD III)
EC 6.3.5.2 GMPS 1 reactions