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This Concept Map, created with IHMC CmapTools, has information related to: CPS I Defiency, undetectable Citrulline levels indicates defect in Carbamoyl Phosphate Synthetase I, catabolism of protein results in nitrogen release, glucose drives urea cycle, L-arginine is an activator for the formation of N-acetylglutamate synthase, NH4+ is especially toxic to CNS, NH4+ build up in circulation causes hyperventilation, Unresponsive Infant must restrict nitrogen intake by adhering to low protein diet, argininosuccinate is hydrolyzed to furmarate, ornithine which via ornithine transcarbamoylase, malate is oxidized to oxaloacetate, CO2 decrease causes respiratory alkalosis, liver once alanine is in liver it is transaminated, L-arginine when urea cycle is inhibited becomes an essential amino acid, citrulline transported out of the mitochondrial matrix to cytosol, urea is transported to kidney and eliminated, phenylacetylglutamine functions as an alternative excretory pathway for NH4+, glucose when present creates high insulin: glucagon ratio, plasma ammonium (1250 umol/L) indicates defect in Carbamoyl Phosphate Synthetase I, oxaloacetate is transaminated to aspartate, NH4+ are unable to combine to form Carbamoyl phosphate