A 3 year old child had a severe viral illness, a few days later there were repeated vomiting, loss of consciousness and convulsions. Laboratory blood tests revealed hyperammonemia.

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