Committed step of purine synthesis; feedback-inhibited by AMP/GMP; azaserine site
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 2.4.2.14 PPAT 1 reactions
EC 1.11.1.9 GPX1 1 reactions
Selenium-dependent; principal RBC peroxide defence
EC 1.8.1.7 GSR 1 reactions
FAD/riboflavin-dependent; links GSH recycling to the pentose phosphate pathway
EC 6.3.2.3 GSS 1 reactions
EC 2.7.1.30 GK 1 reactions
EC 2.7.1.30 / 1.1.1.8 GK / GPD1 1 reactions
EC 2.3.1.15 GPAM 1 reactions
EC 2.4.1.18 GBE1 1 reactions
Andersen disease (GSD IV): polyglucosan, cirrhosis
EC 2.4.1.25 AGL 1 reactions
Cori-Forbes disease (GSD III)
EC 3.2.1.33 AGL 1 reactions
Cori-Forbes disease (GSD III)
EC 2.4.1.1 PYGM / PYGL 1 reactions
McArdle disease (GSD V, muscle); Hers disease (GSD VI, liver)
EC 2.4.1.11 GYS1/GYS2 1 reactions
GSD 0: low glycogen, fasting ketotic hypoglycaemia
EC 2.4.1.186 GYG1 1 reactions
EC 1.1.1.27 LDHA/LDHB 1 reactions
Regenerates NAD+ anaerobically; LDHA deficiency causes exercise-induced myoglobinuria
EC 6.3.5.2 GMPS 1 reactions
EC 3.5.4.3 GDA 1 reactions
1 reactions
EC 1.14.14.18 HMOX1 1 reactions
Produces biliverdin, CO and Fe2+; rate-limiting for heme catabolism
EC 2.7.1.3 KHK 1 reactions
GCK mutations: MODY2; HK1 deficiency: haemolytic anaemia
1 reactions