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This Concept Map, created with IHMC CmapTools, has information related to: B4 CPS1 Deficiency, muscle tissue as alanine, citrulline resulting in undetectable citrulline levels, ornithine transported into mitochondrial matrix in exchange for citrulline via ornithine transporter 1, liver to produce glutamate, N-acetylglutamate allosterically activates carbamoyl phosphate synthetase 1, low-protein diet to reduce amino acid degradation, Unresponsive Infant shortly after birth, begins amino acid degradation, bloodstream filtered by kidneys, phenylacetylglutamine which is excreted in urine, substrates for TCA cycle for energy production, liver and undergoes transamination to form pyruvate, normal orotic acid in urine diagnostically rules out the accumulation of carbamoyl phosphate, glutamine to the liver, ornithine transporter 1 returning ornithine, citrulline which leaves mitochondria and combines with aspartate, amino acid degradation and must rid the body of excess ammonia, oxaloacetate which can be used as substrates for gluconeogenesis, Unresponsive Infant presents with elevated plasma ammonium (1250 umol/L), arginine hydrolytically cleaved by arginase, Unresponsive Infant presents with normal orotic acid in urine