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17 Questions

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Urea Cycle Regulation

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Questions and Answers

In which clinical condition is ammonia level within normal range, while urea level increases in the blood?

Hyperammonemia

Hepatic failure

Renal failure (correct)

Portal hypertension

What is the consequence of ammonia accumulation in the brain on the TCA cycle?

Stimulation of the urea cycle

Debilitation of energy production (correct)

Increase in energy production

No effect on energy production

What is the role of glutamate dehydrogenase enzyme in the brain?

Regulating the urea cycle

Activating N-acetyl glutamate

Converting glutamate to α-ketoglutarate acid

Reacting ammonia with α-ketoglutarate acid to form glutamate (correct)

What is the consequence of collateral circulation in liver cirrhosis?

Ammonia entry into systemic circulation Signup and view all the answers

What is the primary source of energy for the brain?

TCA cycle Signup and view all the answers

What is the primary location where ammonia is converted into non-toxic urea?

Liver Signup and view all the answers

Which enzyme requires N-acetyl glutamate as an activator in the urea cycle?

Carbamoyl phosphate synthase I Signup and view all the answers

What is the immediate product of the reaction between CO2 and ammonia in the urea cycle?

Carbamoyl phosphate Signup and view all the answers

What is the rate-limiting enzyme in the urea cycle that is allosterically activated by N-acetyl glutamate?

Carbamoyl phosphate synthase 1 Signup and view all the answers

What is a potential cause of hyperammonemia?

Deficiency in carbamoyl phosphate synthase I Signup and view all the answers

What is the source of CO2 needed for the first step of the urea cycle?

Citric acid cycle (TCA) Signup and view all the answers

What is the TCA cycle intermediate produced in the urea cycle after the deamination of glutamate?

a-Ketoglutarate Signup and view all the answers

What is the result of a defect in the ornithine transporter in the urea cycle?

All of the above Signup and view all the answers

How many ATP molecules are required for the first step of the urea cycle, catalyzed by carbamoyl phosphate synthase I?

2 Signup and view all the answers

What is the role of N-acetyl glutamate in the urea cycle?

Allosteric activator of carbamoyl phosphate synthase 1 Signup and view all the answers

What is the consequence of liver cell dysfunction on the urea cycle?

Acquired hyperammonemia Signup and view all the answers

What is the relationship between the urea cycle and the TCA cycle?

The TCA cycle produces CO2 and oxaloacetate for the urea cycle Signup and view all the answers