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This Concept Map, created with IHMC CmapTools, has information related to: Two Infants II, AMP activates Glycogen phosphorylase, glucagon/insulin ratio high levels increase cAMP, fructose 1-6 bisphosphate Dephosphorylated by fructose 1,6 bisphosphatase, DHAP converted to fructose 1-6 bisphosphate, Baby 1 Presents Hyperuricemia, Pyruvate is interconverted to lactate, oxaloacetate is phosphorylated to PEP, fructose builds up resulting in Reducing sugar in urine, adenosine metabolized to Uric Acid, autosomal recessive on locus 9q22.3, F-1-P monopolizes free inorganic phosphate, gluconeogenesis is inhibited in the liver due to build up of glucose 6-phosphate, gluconeogenesis inhibition of leads to 20 mg/dL Hypoglycemia, Glyceraldehyde 3 phosphate converted through glycolysis Pyruvate, NADH/NAD+ratio when high, stimulates lactate dehydrogenase, isocitrate dehydrogenase enzyme of TCA cycle, high AMP/ATP ratio inhibits gluconeogenesis, glucose-6-Phosphatase deficiency has two forms 1a, Baby 2 presents 20 mg/dL Hypoglycemia, Baby 1 Presents Reducing sugar in urine