Formation of Carbamoyl Phosphate in Mitochondria Quiz
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Questions and Answers

In which part of the cell is carbamoyl phosphate generated?

  • Golgi apparatus
  • Mitochondria (correct)
  • Endoplasmic reticulum
  • Cytosol
  • What is the role of ornithine carbamoyl transferase?

  • To transfer the carbamoyl group to ornithine (correct)
  • To catalyze the urea cycle
  • To generate carbamoyl phosphate
  • To break down citrulline
  • What is the byproduct of the reaction catalyzed by carbamoyl phosphate synthetase I?

  • Pi
  • ADP
  • CO2
  • ATP (correct)
  • What is the end product of the reaction catalyzed by ornithine carbamoyl transferase?

    <p>Citrulline</p> Signup and view all the answers

    Where does ornithine come from before entering the mitochondria?

    <p>Cytosol</p> Signup and view all the answers

    What is the nature of the reaction catalyzed by carbamoyl phosphate synthetase I?

    <p>Irreversible</p> Signup and view all the answers

    What is the result of the deficiency of homogentsatic oxygenase?

    <p>Alcaptonuria</p> Signup and view all the answers

    What is the effect of lack of melanin in albinos?

    <p>Increased sensitivity to sunlight</p> Signup and view all the answers

    What is the age group most affected by Parkinson's disease?

    <p>People over 60 years old</p> Signup and view all the answers

    What is the result of the deficiency of p-OH phenyl pyruvate oxygenase/ hydroxylase?

    <p>Tyrosinaemia</p> Signup and view all the answers

    What is the effect of the lack of pigment in the eye in albinos?

    <p>Photophobia</p> Signup and view all the answers

    What is the result of the oxidation of homogentisate?

    <p>Formation of a dark pigment</p> Signup and view all the answers

    What is the primary reason behind the abnormal development of the foetus in pregnant women with high phenylalanine levels?

    <p>Accumulation of phenylalanine</p> Signup and view all the answers

    What effect can dietary control of phenylalanine levels have on preventing mental retardation?

    <p>Prevents mental retardation</p> Signup and view all the answers

    In the context of phenylalanine accumulation, what might happen to the transport of other amino acids to the brain?

    <p>Inhibited transport</p> Signup and view all the answers

    Which amino acid disorder is specifically linked with poor psychomotor maturation, hypopigmentation, and seizures?

    <p>Phenylketonuria</p> Signup and view all the answers

    What is the key role of tyrosine transaminase deficiency in tyrosinaemia?

    <p>Causes excessive accumulation of tyrosine and its metabolites</p> Signup and view all the answers

    What is the shared clinical manifestation between tyrosinaemia and phenylketonuria?

    <p>Hypopigmentation</p> Signup and view all the answers

    What is the enzyme responsible for catalyzing the synthesis of citrulline in the mitochondria?

    <p>Citrulline synthetase</p> Signup and view all the answers

    Which molecule provides the second amino group needed for the formation of urea?

    <p>Aspartate</p> Signup and view all the answers

    What from ATP is cleaved during the condensation of aspartate and citrulline to form arginino succinate?

    <p>ATP</p> Signup and view all the answers

    Which of the following is formed during the hydrolysis of PPi by pyrophosphatase?

    <p>Inorganic phosphate</p> Signup and view all the answers

    What is the final product from the condensation of aspartate and citrulline?

    <p>Arginine</p> Signup and view all the answers

    What is the driving factor for the condensation of aspartate and citrulline in the formation of arginino succinate?

    <p>ATP hydrolysis</p> Signup and view all the answers

    Which enzyme is deficient in Maple Syrup Urine Disease?

    <p>α-keto acid dehydrogenase</p> Signup and view all the answers

    What is the primary cause of brain dysfunction in Maple Syrup Disease?

    <p>High leucine levels</p> Signup and view all the answers

    Which amino acids are affected in Maple Syrup Urine Disease?

    <p>Leucine, Isoleucine, Valine</p> Signup and view all the answers

    What symptoms might be observed in an individual with Maple Syrup Disease?

    <p>Lethargy, Hypoglycemia, Convulsions</p> Signup and view all the answers

    What is found in the urine of individuals with Maple Syrup Urine Disease that contributes to its distinct odor?

    <p>α-ketoisocaproic acid</p> Signup and view all the answers

    Which enzyme is affected in Isovaleric Acidemia?

    <p>Isovaleryl-CoA dehydrogenase</p> Signup and view all the answers

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