Urea cycle Flashcards
Step 1
enzyme: carbamoyl phosphate synthetase
purpose: to form carbamoyl phosphate from bicarbonate and ammonia
Step 2
enzyme: ornithine trans-carbamoylase
purpose: to transfer carbamoyl group to orthinine to produce citrulline which is then transported to the cytoplasm
Step 3
enzyme: argininosuccinate synthetase
purpose: to condence citrulline with aspartate to produce arignino-succinate
Step 4
enzyme: argininosuccinase
purpose: to separate asp carbon skeleton by cleaving off fumarate and leaving argining
Step 5
enzyme: arginase
purpose: to hydrolyze arg side chain to produce urea and regenerate ornithine
Ala and gln produced during amino acid breakdown
act as transporters to move excess NH3 to the liver
both are converted to glutamate in the liver by glutaminase or transaminase
NH3 is released as
ammonia
How is the second NH3 group on urea produced
glu can transfer its NH3 to oxaloacetate through a transaminase reaction, producing asp which produces the second NH3 group
How is the urea cycle regulated
- carbamoyl phosphate synthetase is allosterically activated by N-acetylglutamate (NAG)
- Glu buildup occurs during amino acid breakdown which stimulates NAG production. this activates CPSI to dispose of excess amino groups
How is N-acetylglutamate produced
enzyme: N-acetylglutamate synthase
materials needed: acetyl-coa and glutamate
What is the committed step of the urea cycle
step 1