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This Concept Map, created with IHMC CmapTools, has information related to: Biochem Urea Edit, Elevated Glutamine levels (1500umol/L) leads to increased intracerebral ammonium and glutamine, other energy-producing metabolic pathway (glycolysis) leading to decreased catabolism of proteins, Elevated Plasma ammonium (1250umol/L) leads to increased intracerebral ammonium and glutamine, increased intracerebral ammonium and glutamine stimulates hyperventilation, buildup of orotic acid contradicting diagnosis of OTC deficiency, Ornithine transcarbamoylase if defective results in OTC deficiency, hyperventilation decreases respiratory alkalosis (pH = 7.56), normal PO2 consistent with respiratory alkalosis (pH = 7.56), low HCO3- (15 microM/L) consistent with respiratory alkalosis (pH = 7.56), high glucose providing substrate for other energy-producing metabolic pathway (glycolysis), Carbamoyl Phosphate Synthetase I (CPS I) Enzyme which would decrease effects of mutation on remainder of pathway, starting with reduced synthesis of carbamoyl phosphate, buildup of orotic acid consistent with normal orotic acid