30 Questions
What is the result of glucagon binding to its cell-surface receptor?
Activation of adenylate cyclase
What is the substrate for monoacylglycerol lipase?
Monoacylglycerols
What is the role of PKA in the release of fatty acids?
Phosphorylation and activation of hormone-sensitive lipase
What is the net result of the action of hormone-sensitive lipase and other enzymes on triacylglycerols?
Three moles of free fatty acid and one mole of glycerol
What is the function of hormone-sensitive lipase?
Hydrolysis of fatty acids from triacylglycerols
What is the result of the activation of adenylate cyclase?
Increase in cAMP
What is the primary function of enoyl CoA isomerase in β-oxidation?
To move the naturally occurring cis- bond to the trans- bond
What is the consequence of carnitine deficiency in newborns?
Inability to transport fatty acids into the mitochondria for oxidation
Which of the following is a symptom of Carnitine Palmitoyltransferase II (CPT II) deficiency?
Recurrent muscle pain and fatigue
What is the primary organ affected by Carnitine Palmitoyltransferase I (CPT I) deficiency?
Liver
What is the treatment for carnitine deficiency?
Oral administration of carnitine
What is the primary difference between the oxidation of saturated and unsaturated fatty acids?
Unsaturated fatty acids require enoyl CoA isomerase
What is the role of carnitinepalmitoyltransferase I in the transport of fatty acids into the mitochondria?
To exchange CoA for carnitine in the cytoplasm
What is the product of the first step in β-oxidation?
Trans-Δ2-enoyl-CoA
What is the enzyme responsible for the hydration of the double bond in the second step of β-oxidation?
Enoyl-CoA hydratase
What is the product of the third step in β-oxidation?
A ketone
What is the final product of β-oxidation?
Acyl-CoA and a long chain fatty acyl CoA
What is the role of β-ketoacyl-CoA thiolase in β-oxidation?
To catalyze the thiolytic cleavage of the thioester
What is the final product of the β-oxidation of fatty acids with an odd number of carbon atoms?
Succinyl-CoA units
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?
Methylmalonyl CoA racemase
What is the role of enoyl CoA isomerase in the β-oxidation of fatty acids with an odd number of carbons?
To convert cis double bonds to trans double bonds
What is the first step in the β-oxidation of fatty acids with an odd number of carbons?
Carboxylation by the biotin-dependent propionyl CoA carboxylase
What is the product of the ATP-dependent pathway that propionyl-CoA undergoes?
Succinyl-CoA
Why is enoyl CoA isomerase required in the β-oxidation of fatty acids with an odd number of carbons?
Because β-oxidation generates or requires pre-existing double bonds at even-numbered carbons
What is the effect of sulfonylurea drugs on carnitine acyltransferases?
They inhibit the enzyme
What is the most common form of acyl-CoA dehydrogenase deficiency?
Medium Chain Acyl CoA Dehydrogenase Deficiency
What is the diagnostic indicator of Medium Chain Acyl CoA Dehydrogenase Deficiency?
Excessive urinary excretion of medium-chain dicarboxylic acids
What is the primary cause of Refsum's disease?
Deficiency of the mitochondrial α-oxidizing enzyme
What is the primary treatment for Refsum's disease?
Restriction of dairy products and ruminant meat from the diet
What is the typical age range when symptoms of Medium Chain Acyl CoA Dehydrogenase Deficiency become apparent?
Early childhood (1-5 years)
Understand the complex process of hormone-regulated release of metabolic energy in the form of fatty acids. Learn how hormones like glucagon, epinephrine, and β-corticotropin stimulate the activation of hormone-sensitive lipase in adipocytes.
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