Urea Cycle

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Overall Sources & Utilization of Amino Acids

Gamma-glutamyl Cyacle – Meister’s 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

Catabolic Disposition (Fates) of Carbon Chains of Amino Acids


Methionine

Inborn Errors
1. Homocystinuria
- Deficiency of cystathionine
synthetase
2 Cystathioninuria
- Deficiency of cystathionase
Glucose – Alanine Cycle

Synthesis of Non-essential amino acids


1. Glycine Serine
2. Proline Glutamic acid
3. Arginine Glutamic acid
4. Histidine Glutamic acid
5. Tryptophan Alanine
6. Phenylalanine Tyrosine
7. Threonine Glycine
8. Methionine Cysteine
9. Aspartic acid Asparagine
10. Glutamic acid Glutamine
11. Pyruvate Alanine
12. Oxaloacetate Aspartic acid
13. -ketoglutarate Glutamic acid
14. 3-phosphoglycerate Serine

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