Lack of effect of increased 2,3-diphosphoglycerate on flux through the oxidative pathway in phosphoglycerate kinase deficiency.
Erythrocyte enzyme and substrate levels in two subjects with phosphoglycerate kinase deficiency are reported. The effect of the increased 2,3-diphosphoglycerate, which has been reported as an inhibitor of 6-phosphogluconate dehydrogenase, on the flux through the pentose phosphate pathway, was assessed. There was no significant difference in the flux through erythrocytes from one of the affected subjects and normal subjects in the presence and absence of methylene blue.
D019794:2,3-Diphosphoglycerate; D000328:Adult; D000743:Anemia, Hemolytic / Q000201:enzymology; D002648:Child; D004163:Diphosphoglyceric Acids / Q000378:metabolism*; D004912:Erythrocytes / Q000201:enzymology; D006801:Humans; D008297:Male; D008751:Methylene Blue; D010427:Pentose Phosphate Pathway; D010735:Phosphoglycerate Kinase / Q000172:deficiency*
D004163:Diphosphoglyceric Acids; D019794:2,3-Diphosphoglycerate; D010735:Phosphoglycerate Kinase; D008751:Methylene Blue