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

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

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

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

<p>α-ketoglutarate (A)</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 (B)</p> Signup and view all the answers

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

<p>Glutamate and NH3 (D)</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 (B)</p> Signup and view all the answers

What is the clinical significance of transaminases in the blood?

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

Why is early diagnosis of PKU crucial?

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

What is the composition of creatine kinase?

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

What is the byproduct of the deamination reaction?

<p>NH3 (A)</p> Signup and view all the answers

What is the normal blood concentration of ammonia?

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

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

<p>Fatal (D)</p> Signup and view all the answers

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

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

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

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

What is the enzyme involved in the transamination reaction?

<p>Transaminase (B)</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 (C)</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 (A)</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 (A)</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 (D)</p> Signup and view all the answers

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

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

What is the toxic compound that accumulates in phenylketonuria?

<p>Phenyl pyrovic acid (D)</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 (B)</p> Signup and view all the answers

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