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This Concept Map, created with IHMC CmapTools, has information related to: CPS I defect, Urine of Unresponsive Infant, Oxaloacetate converted to L-aspartate, Malate converted by TCA cycle to Oxaloacetate, citrulline in cytosol combine to form argininosuccinate, Unresponsive Infant consumes protein in diet, pyruvate enters TCA cycle, Fumarate converted to Malate, NH4+ accumulation leads to anion gap metabolic acidosis, Glutamine combines with sodium phenylacetate to phenylacetylglutamine, Ornithine in cytosol transported into mitochondria Ornithine in mitochondria, Unresponsive Infant treated with oral arginine, anion gap metabolic acidosis patient responds with compensatory respiratory alkalosis, Urea excreted in Urine, TCA cycle eventually converts pyruvate to Malate, L-arginine converted by arginase to form Ornithine in cytosol, protein in diet converted to NH3, oral arginine leads to the eventual increase in concentration of substrates of CPS-I ,via Le Chatelier's principle increases activity of CPS-I, CPS-I when defected lead to increase in NH3, CPS-I adds NH3 to CO2 to form Carbamoyl phosphate, Unresponsive Infant treated with sodium phenylacetate