Contents 1 Case 15: Urea Cycle 1.1 1. Describe the function of the urea cycle. 1.2 2. List the tissue (liver) and sub-cellular compartments (mitochondrial matrix and cytosol) where the pathway is located. 1.3 3. Draw the urea cycle. 1.4 4. Describe how the nitrogen atoms of urea can be derived from all 20 amino acids. 1.5 5. Describe the regulation of the urea cycle and name the rate-limiting step in the cycle. 1.6 6. List three consequences of hyperammonemia. 1.7 7. Explain how protein metabolism during fasting contributes to the urea cycle. 1.8 8. Compare and contrast deficiencies in different enzymes that result in urea cycle defects. 1.9 9. Describe newborn screening for metabolic disorders.

[edit] Case 15: Urea Cycle

[edit] 1. Describe the function of the urea cycle.

[edit] 2. List the tissue (liver) and sub-cellular compartments (mitochondrial matrix and cytosol) where the pathway is located.

[edit] 3. Draw the urea cycle.

[edit] 4. Describe how the nitrogen atoms of urea can be derived from all 20 amino acids.

[edit] 5. Describe the regulation of the urea cycle and name the rate-limiting step in the cycle.

[edit] 6. List three consequences of hyperammonemia.

[edit] 7. Explain how protein metabolism during fasting contributes to the urea cycle.

[edit] 8. Compare and contrast deficiencies in different enzymes that result in urea cycle defects.

[edit] 9. Describe newborn screening for metabolic disorders.