Abstract
Hexose monophosphate was measured in the blood of 2 patients with glycogenstorage disease Type I, 6 patients with glycogen-storage disease not of Type I, and 5 normals. The hexose monophosphate levels in the 2 patients with glycogen-storage disease Type I were lower than in all other persons investigated. In the blood of one of these 2 patients, however, a 13.4-fold increase in pyruvate and a 14.6-fold increase in lactate was found, compared with the mean values in 8 normals. The total amount of phosphorylated glycogenolytic intermediates (1,3-diphosphoglycerate was not included in this assay) in the blood of this patient was insignificant.
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