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This Concept Map, created with IHMC CmapTools, has information related to: urea synthesis, alpha-ketogluterate produces glutamate, NH3 provides nitrogen for reaction with CO2 and 2ATP, low levels of available gluconeogenic amino acids reduces gluconeogenesis, NH3 combine to form glutamine, oxaloacetate to produce aspartate, CNS toxicity (neuro-toxic effects) leads to unresponsive infant, NH3 provides nitrogen for reaction with CO2 and 2ATP, treatments including L-arginine infusion, Arginase to produce ornithine in cytosol, glutamate converted by glutamine synthetase, unresponsive infant testing reveals elevated plasma ammonium (1250mmol/L), glutamate combine to form glutamine, treatments including glucose administration, ALT to transfer amino groups from alanine, alanine to alpha-ketogluterate, unresponsive infant has a autosomal recessive mutation, glutamine synthetase to produce glutamine for nontoxic transport of ammonia, carbamoyl phosphate in the mitchondria matrix disrupts the conversion of ornithine in mito matrix by ornithine transcarbamoylase, treatments including sodium phenylacetate, carbamoyl phosphate in the mitchondria matrix is not available as substrate for the synthesis of orotic acid