Urea Cycle
Urea Cycle
Urea Cycle
3 Requirements:
1) membrane-bound enzyme
(Gamma glutamyl transpeptidase)
2) a mol of GSH
3) 3 moles of ATP
Central Role of the Liver in Amino Acid Metabolism
Urea Cycle
Enzymes:
1. Carbamoyl PO4 synthetase
2. Ornithine transcarbamoylase
3. Argininosuccinate synthetase
4. Argininosuccinase
5. Arginase
Metabolic Pathway of Phe/Tyr
Enzymes:
1. Phenylalanine
monooxygenase or
phenylalanine oxidase
or phenylalanine
hydroxylase
2. Homogentisate 1,2- dioxygenase
3. Tyrosinase
Inborn Errors
1. Homocystinuria
- Deficiency of cystathionine
synthetase
2 Cystathioninuria
- Deficiency of cystathionase
Glucose – Alanine Cycle