urea cycle Flashcards
what transports NH4 in the bloodstream
glutamine
glutamine synthase
cataylyzes the combination of free ammn=onia with glutamate to yield glutamine
requires ATP
-is critical to transport toxic ammonia to the liver
glutaminase
catalyzes the conversion of glutamine to glutamate and NH4
glycose alanine cycle
skeletal muscles produce pyruvate, lactate, and ammonia
alanine carries ammonia and carbon skeleton from pyruvate to liver
-ammonia is excreted
-pyruvate is used to produce glucose which is returned to muscle
alanine aminotransferase
intervonverts pyruvate and alanine via transamination with glutamate
five enzymatic steps of urea production form ammonia
- carbamoyl phosphate synthetase I
- ornithine trascarbamoylase
- agrininosuccinate synthase
- argininosuccinase
- arginase
carbamoyl phsphate synthetase I
brings in ammonia from deamination reactions
ornithine trascarbamoylase
brings carbamoyl phsophate into the urea cycle resulting in citrulline which can enter the cytosisol
also forms Pi from ornithine and carbamoyl phosphate
argininosuccinate synthetase
brings in the second amino group from aspartate
catalyzes the condensation of the amino group of aspartate and the ureido group of citrulline
-requires ATP
-uses an intermediate
argininosuccinase
removes length of carbon skeleton as fumarate
catalyzes reversible cleavage of argininosuccinate from arginine and fumarate
-fumarate is converted to malate and joins the pool of cirtic acid cycle intermedites
arginase
cleavage of arginine to form urea and ornithine - back at begining of cycle
-ornithine formed is transported into mitochondrion to initiate another round
rate limiting step of urea cycle
carbamoyl phsophate sythetase I reaction
-has two activation steps that require ATP
-second step of reaction uses ammonia from deamination reaction
what happens during the formation of argininosuccinate
cirtrulline passes from mitochondrion to cytosol
aspartate arginosuccinate shunt
pathways linking the citric acid cycle and urea cycle
-link the fates of the amino groups and the carbon skeletons of amino groups
regulation of the activity of the urea cycle
occurs at two levels
1. level of enzyme synthesis for urea cycle enzymes and carbamoyl phosphate synthetase I
2. allosteric regulation of carbamoyl phosphate syntehtase I