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This Concept Map, created with IHMC CmapTools, has information related to: B6 UCD, Phenylacetylglutamine as a mechanism to excrete Nitrogen, L-Ornithine (cytosol) transported by ORNT1, Elevated Orotic Acid suggests Defect in OTC, ORNT1 to L-Ornithine (mitochondrial matrix, CO2 leading to Respiratory Alkalosis, Hyperammonemia treated with Glucose (intravenous), Aminotransferase catalyzes Oxaloacetate, Blue Line is Treatment, Hyperammonemia treated with Sodium Phenylacetate (intrevenous), L-Ornithine (mitochondrial matrix catalyzed by Ornithine Transcarbamoylase, High Glutamine Plasma Levels (1500 mMol/L) in Patient, L-Arginine (intravenous) becomes Essential Amino Acid, The Color Key is Enzymes, Arginine required for synthesis of N-Acetylglutamate, Glutamine (tissues) catalyzed by Glutaminase, UREA excreated in Urine, Essential Amino Acid promotes formation of Citruline, Oxaloacetate into a-Ketogluterate, Sodium Phenylacetate (intrevenous) combines with Glutamine (contains 2N), L-Citruline (cytosol catalyzed by Argininosuccinate Synthetase