what is it hyperammonemia? when there is hyperammonemia? Inc^ NH3 in blood Metabolic fate of ammonia. Formation of urea, amination of ketoacid, Glutamate to glutamine, ketogluterate to glutamate Transport of ammonia. NH3+Glutamate -> glutamine -> glutamate +NH3--->urea cycle (tissue blood liver) Reactions of urea cycle. Significance of urea cycle. Dec^ NH3 and make it safe for excretion Regulation of urea cycle . Carbonyl phosphate syanthesis-> rate controling enzyme Metabolic inborn errors of urea cycle 6 enzymes of the cycle CPS1, NAGS, OTC, AS, ASL, AG
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