Dai 2 Flashcards
_____, a cofactor of aminotransferase, is a derivative of vitamin __.
PLP Pyridoxal phosphate, B6
transamination step: aketoglutarate to glutamate by enzyme _______.
oxidative deamination step: glutamate to NH3 by enzyme ______
aminotransferase
glutamate dehydrogenase.
Decreased arginine, citrulline, urea, and increased orotic aciduria is a sign of
OTC deficiency
CPS1 is located in the ____
CPS2 is located in the ____
mitochondria
cytosol
CP can spill into cytosol and become pyrimidines during _____deficiency.
OTC
deficiency of arginosuccinate synthetase leads to accumulation of
citrulline
deficiency of arginosuccinate lyase leads to accumulation of
arginosuccinate,
arginase deficiency leads to accumulation of
arginine.
in differential diagnosis of urea cycle disorders in neonates, no citrulline and no orotic acid are signs of
CPS1 or NAG synthase deficiency.
CPS1 deficiency is an autosomal _________ disorder.
OTC deficiency is an ____________ disorder
recessive
xlinked
in urea cycle deficient patients, a low N diet can be supplemented with _________
a keto acid, can become aa via aminotransferase.
T or F. alternative n excretion: ammonia can react with Glycine+THF, and glutamate and be excreted.
true
T or F. arginine is a substrate and positive regulator of urea cycle.
true. arginine supplementatoin can help urea cycle disorders, unless its an arginase deficiency.
hydrolysis of glutamine by glutaminase generates nh3 that is an important defense mechanism against _____.
acidosis. good for places that arent liver kidney,
Name a few other alternative sources of ammonia besides aa degradation.
glutaminase
bacterial urease
direct deamination of certain aa.
