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This Concept Map, created with IHMC CmapTools, has information related to: 2.23 Inborn Errors of Urea Synthesis, decreased upregulation of N-acetyglutamate synthetase preventing allosteric activation of defective Carbamoyl phosphate synthetase I (CPS I), arterial blood gas analysis revealed respiratory alkalosis (pH = 7.56), Unresponsive infant lab tests included plasma analysis, Unresponsive infant treated with high glucose, urinalysis revealed normal orotic acid, NH3 produced from breakdown of glutamate, ammonia decreasing need for defective Carbamoyl phosphate synthetase I (CPS I), citrulline consistent with undetectable citrulline levels, glutamine leading to increased glutamine excretion in the urine, Unresponsive infant treated with L-arginine infusion, reduced synthesis of carbamoyl phosphate preventing buildup of oronic acid, Unresponsive infant lab tests included arterial blood gas analysis, mutated CPS I gene resulting in defective Carbamoyl phosphate synthetase I (CPS I), buildup of oronic acid consistent with normal orotic acid, blood leukocyte DNA analysis revealed mutated CPS I gene, reduced synthesis of carbamoyl phosphate prevents reaction with ornithine, Unresponsive infant presents with poor feeding, Unresponsive infant treated with Low protein diet, reduced synthesis of carbamoyl phosphate from CO2, L-Aspartate normally a source of nitrogen for urea