Y1S2 005 III Biochem Hormone Regulation of Metabolic Energy Release
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Questions and Answers

What is the result of glucagon binding to its cell-surface receptor?

  • Inhibition of hormone-sensitive lipase
  • Release of glycerol
  • Activation of adenylate cyclase (correct)
  • Phosphorylation of diacylglycerol lipase
  • What is the substrate for monoacylglycerol lipase?

  • Triacylglycerols
  • Monoacylglycerols (correct)
  • Fatty acids
  • Diacylglycerols
  • What is the role of PKA in the release of fatty acids?

  • Phosphorylation of diacylglycerol lipase
  • Inhibition of adenylate cyclase
  • Phosphorylation and activation of hormone-sensitive lipase (correct)
  • Release of glucagon
  • What is the net result of the action of hormone-sensitive lipase and other enzymes on triacylglycerols?

    <p>Three moles of free fatty acid and one mole of glycerol</p> Signup and view all the answers

    What is the function of hormone-sensitive lipase?

    <p>Hydrolysis of fatty acids from triacylglycerols</p> Signup and view all the answers

    What is the result of the activation of adenylate cyclase?

    <p>Increase in cAMP</p> Signup and view all the answers

    What is the primary function of enoyl CoA isomerase in β-oxidation?

    <p>To move the naturally occurring cis- bond to the trans- bond</p> Signup and view all the answers

    What is the consequence of carnitine deficiency in newborns?

    <p>Inability to transport fatty acids into the mitochondria for oxidation</p> Signup and view all the answers

    Which of the following is a symptom of Carnitine Palmitoyltransferase II (CPT II) deficiency?

    <p>Recurrent muscle pain and fatigue</p> Signup and view all the answers

    What is the primary organ affected by Carnitine Palmitoyltransferase I (CPT I) deficiency?

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

    What is the treatment for carnitine deficiency?

    <p>Oral administration of carnitine</p> Signup and view all the answers

    What is the primary difference between the oxidation of saturated and unsaturated fatty acids?

    <p>Unsaturated fatty acids require enoyl CoA isomerase</p> Signup and view all the answers

    What is the role of carnitinepalmitoyltransferase I in the transport of fatty acids into the mitochondria?

    <p>To exchange CoA for carnitine in the cytoplasm</p> Signup and view all the answers

    What is the product of the first step in β-oxidation?

    <p>Trans-Δ2-enoyl-CoA</p> Signup and view all the answers

    What is the enzyme responsible for the hydration of the double bond in the second step of β-oxidation?

    <p>Enoyl-CoA hydratase</p> Signup and view all the answers

    What is the product of the third step in β-oxidation?

    <p>A ketone</p> Signup and view all the answers

    What is the final product of β-oxidation?

    <p>Acyl-CoA and a long chain fatty acyl CoA</p> Signup and view all the answers

    What is the role of β-ketoacyl-CoA thiolase in β-oxidation?

    <p>To catalyze the thiolytic cleavage of the thioester</p> Signup and view all the answers

    What is the final product of the β-oxidation of fatty acids with an odd number of carbon atoms?

    <p>Succinyl-CoA units</p> Signup and view all the answers

    Which enzyme is responsible for converting the D-isomer to the L-isomer in the β-oxidation of fatty acids with an odd number of carbons?

    <p>Methylmalonyl CoA racemase</p> Signup and view all the answers

    What is the role of enoyl CoA isomerase in the β-oxidation of fatty acids with an odd number of carbons?

    <p>To convert cis double bonds to trans double bonds</p> Signup and view all the answers

    What is the first step in the β-oxidation of fatty acids with an odd number of carbons?

    <p>Carboxylation by the biotin-dependent propionyl CoA carboxylase</p> Signup and view all the answers

    What is the product of the ATP-dependent pathway that propionyl-CoA undergoes?

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

    Why is enoyl CoA isomerase required in the β-oxidation of fatty acids with an odd number of carbons?

    <p>Because β-oxidation generates or requires pre-existing double bonds at even-numbered carbons</p> Signup and view all the answers

    What is the effect of sulfonylurea drugs on carnitine acyltransferases?

    <p>They inhibit the enzyme</p> Signup and view all the answers

    What is the most common form of acyl-CoA dehydrogenase deficiency?

    <p>Medium Chain Acyl CoA Dehydrogenase Deficiency</p> Signup and view all the answers

    What is the diagnostic indicator of Medium Chain Acyl CoA Dehydrogenase Deficiency?

    <p>Excessive urinary excretion of medium-chain dicarboxylic acids</p> Signup and view all the answers

    What is the primary cause of Refsum's disease?

    <p>Deficiency of the mitochondrial α-oxidizing enzyme</p> Signup and view all the answers

    What is the primary treatment for Refsum's disease?

    <p>Restriction of dairy products and ruminant meat from the diet</p> Signup and view all the answers

    What is the typical age range when symptoms of Medium Chain Acyl CoA Dehydrogenase Deficiency become apparent?

    <p>Early childhood (1-5 years)</p> Signup and view all the answers

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