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Additionally, the Pcca -/- (A138T) mouse model, representing one of the gene mutations observed in human patients with a milder phenotype 38,49,56,57 , may not fully recapitulate the diverse conditions (various genotypes, disease severity, and metabolic crises) encountered in human PA patients

Conner, JD

The developing brain is much more susceptible to the harmful effects of ammonia than a mature brain.11 Its toxicity is associated to the elevation of extracellular glutamate levels and the development of cerebral oedema with increased intracranial pressure.4 The urea cycle is the main metabolic pathway to eliminate ammonia, transforming it into urea that is later excreted by the kidney

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2021 Nov;20(11)

Muscle Performance Lysine is a precursor of carnitine that is involved in fatty acid oxidation in mitochondria and, like arginine and some other amino acids, stimulates growth hormone release and its concentrations in plasma [97]