Urea Cycle Reactants and Enzymes Quiz

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Questions and Answers

Where does the first reaction of the urea cycle take place?

  • Golgi apparatus
  • Mitochondria (correct)
  • Endoplasmic reticulum
  • Cytosol

What is the activator of Carbamoyl phosphate synthetase I (CPS I)?

  • N-Acetyl-Glutamate (correct)
  • Carbamoyl phosphate
  • Ornithine
  • Argininosuccinate

Which syndrome is caused by the mutation of the ornithine transport gene?

  • Huntington's disease
  • HHH syndrome (correct)
  • Marfan syndrome
  • Turner syndrome

What is the product formed when citrulline reacts with 1 more ammonia from Aspartic acid in the cytosol?

<p>Argininosuccinate (B)</p> Signup and view all the answers

Why do we still need to get ornithine from the diet if it is immediately converted to urea?

<p>For protein synthesis (B)</p> Signup and view all the answers

Which enzyme catalyzes the conversion of arginine to urea and ornithine in the urea cycle?

<p>Arginase (B)</p> Signup and view all the answers

What is the immediate consequence of the deficiency of any enzyme in the urea cycle?

<p>Hyperammonemia (B)</p> Signup and view all the answers

Which step in the urea cycle involves the detoxification of 1 mole of ammonia?

<p>1st reaction catalyzed by CPS I (C)</p> Signup and view all the answers

Which molecule is NOT produced as a direct result of the urea cycle?

<p>Fumarate (C)</p> Signup and view all the answers

What is the total amount of ATP consumed in both the 1st and 3rd reactions of the urea cycle?

<p>4 moles of ATP (A)</p> Signup and view all the answers

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