Lipid Metabolism and Transport
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Questions and Answers

What is the normal concentration of ketone bodies in the circulation?

  • 1mM (correct)
  • 10mM
  • 0.1mM
  • 100mM

    • What is required for the synthesis of ketone bodies?

  • Fatty acids available for oxidation in the liver (correct)
  • Low plasma insulin only
  • High plasma insulin/glucagon ratio
  • Fatty acids available for oxidation in the brain

    • What is the role of ketone bodies in the body?

  • They are used by all tissues containing mitochondria (correct)
  • They are used only by the liver and kidneys
  • They are not used by any tissues
  • They are used only by the central nervous system

    • What is the primary component of cell membrane?

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

    What is the consequence of a fall in plasma insulin?

    <p>It activates the lyase and inhibits the reductase</p> Signup and view all the answers

    Where does lipolysis occur in the process of ketone body synthesis?

    <p>In adipose tissue</p> Signup and view all the answers

    What is the primary function of the enzymes found in the liver and kidney?

    <p>To remove the NH2-group from amino acids</p> Signup and view all the answers

    What is the usual keto-acid involved in the transamination reaction?

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

    What is the product of the reaction between glutamic acid and pyruvic acid?

    <p>Alanine and α-keto glutarate</p> Signup and view all the answers

    What is the product of the reaction between glutamine and glutaminase?

    <p>Glutamate and NH3</p> Signup and view all the answers

    What is the purpose of detoxifying ammonia in the body?

    <p>To prevent the build-up of toxic compounds</p> Signup and view all the answers

    What is the clinical significance of transaminases in the blood?

    <p>To assess liver function</p> Signup and view all the answers

    Why is early diagnosis of PKU crucial?

    <p>To prevent irreversible damage</p> Signup and view all the answers

    What is the composition of creatine kinase?

    <p>Two subunits of different types (B and M)</p> Signup and view all the answers

    What is the byproduct of the deamination reaction?

    <p>NH3</p> Signup and view all the answers

    What is the normal blood concentration of ammonia?

    <p>Low, around 25-40μM</p> Signup and view all the answers

    What is the consequence of a complete loss of an enzyme in the urea cycle?

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

    Which of the following is NOT a transaminase of clinical importance?

    <p>Glutamate transaminase (GT)</p> Signup and view all the answers

    What is the primary purpose of a low-protein diet in the treatment of urea cycle defects?

    <p>To restrict the amount of amino acid in the diet</p> Signup and view all the answers

    What is the source of ammonia produced during amino acid degradation?

    <p>Gut bacteria</p> Signup and view all the answers

    What is the enzyme involved in the transamination reaction?

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

    What is the molecular basis of the toxic effect of ammonia?

    <p>Its reaction with alpha-ketoglutarate to form glutamate</p> Signup and view all the answers

    What is the purpose of replacing amino acids with keto acids in the treatment of urea cycle defects?

    <p>To restrict the amount of amino acid in the diet</p> Signup and view all the answers

    How is PKU typically diagnosed in newborns in the UK?

    <p>Through screening for phenylketones in urine and phenylalanine in blood</p> Signup and view all the answers

    Which of the following amino acids is essential and cannot be totally removed from the diet?

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

    What is the reaction catalyzed by creatine kinase in muscle cells?

    <p>Creatine + ATP → Creatine Phosphate + ADP</p> Signup and view all the answers

    What is the consequence of elevated levels of homocysteine and methionine in plasma and the presence of homocystine in the urine?

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

    What is the significance of the amount of creatinine in blood and urine?

    <p>Indicates kidney function and muscle turnover</p> Signup and view all the answers

    What is the toxic compound that accumulates in phenylketonuria?

    <p>Phenyl pyrovic acid</p> Signup and view all the answers

    What is the significance of the three different enzymes of creatine kinase?

    <p>They are isoenzymes with different tissue distributions</p> Signup and view all the answers

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