| Modification Notes |
PGD (phosphogluconate dehydrogenase), also referred to as 6PGD, is a 483 amino acid enzyme that is involved in the pentose phosphate shunt. Pentose is required for nucleic acid biosynthesis and the pentose phosphate cycle is a major source of NADPH. As the second dehydrogenase in the pentose phosphate cycle, PGD catalyzes the oxidative decarboxylation of PGD to ribulose 5-phosphate, following the release of CO2 and the reduction of NADP. PGD deficiency increases the level of erythrocyte pyruvate kinase (PK) activity and reduces glutathione synthetase (GSH), resulting in hemolysis. Defects in PGD are generally asymptomatic and are inherited in an autosomal dominant fashion. Catalytic active regions of PGD, such as those forming the substrate and coenzyme binding sites, are highly conserved in most species. |