Fatty Acid Transport into Mitochondria

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What is the suffix commonly used in the nomenclature of fatty acids?

-ene

-ic (correct)

-yne

-ane

What is the name of the C18 saturated fatty acid derived from the parent hydrocarbon Octadecane?

Octadecanoic Acid (correct)

Octadecadienoic Acid

Octadecenoic Acid

Octadecatrienoic Acid

Where do fatty acid molecules undergo breakdown to generate Acetyl-CoA for the Krebs Cycle?

Mitochondria and peroxisomes (correct)

Peroxisomes and cytoplasm

Endoplasmic reticulum and lysosomes

Golgi apparatus and nucleus

What is the primary source of fatty acids for oxidation?

Diet and mobilization from cellular stores Signup and view all the answers

Where are fatty acids stored in the body?

Adipocytes of adipose tissue Signup and view all the answers

What triggers the mobilization of fatty acids from stored triacylglycerols?

Increase in energy demands Signup and view all the answers

What is the primary function of insulin in adipose tissue?

To inhibit the mobilization of fat from adipose tissue Signup and view all the answers

Which enzyme is responsible for activating fatty acids in the cytoplasm?

Fatty acyl-CoA ligase Signup and view all the answers

What is the net result of the fatty acid activation process?

The consumption of 2 molar equivalents of ATP Signup and view all the answers

Where does the oxidation of fatty acids occur?

In the mitochondria Signup and view all the answers

What is the intermediate molecule involved in the transport of fatty acyl-CoA into the mitochondria?

Acyl-carnitine Signup and view all the answers

What is the function of carnitine acyltransferase II?

To catalyze the regeneration of fatty acyl-CoA Signup and view all the answers

What is the consequence of high concentrations of acetyl CoA on β-oxidation?

It inhibits β-oxidation Signup and view all the answers

What is the primary regulator of β-oxidation as a whole?

Fatty acid availability Signup and view all the answers

How many moles of NADH are generated per round of β-oxidation?

1 mole Signup and view all the answers

What happens to the NADH and FADH2 produced during β-oxidation?

They enter the respiratory pathway for the production of ATP Signup and view all the answers

Why do fatty acids yield more energy per carbon atom than carbohydrates?

Because fatty acids produce more ATP per carbon atom Signup and view all the answers

What is the net result of the oxidation of one mole of oleic acid?

146 moles of ATP Signup and view all the answers

Which of the following drugs can inhibit carnitine acyltransferases?

Tolbutamide Signup and view all the answers

Which group of inherited diseases impairs β-oxidation due to deficiencies in acyl-CoA dehydrogenases?

Inherited diseases of acyl-CoA dehydrogenase deficiency Signup and view all the answers

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

Medium Chain Acyl CoA Dehydrogenase Deficiency Signup and view all the answers

Excessive urinary excretion of which compounds is diagnostic of Medium Chain Acyl CoA Dehydrogenase Deficiency?

Medium-chain dicarboxylic acids Signup and view all the answers

Which rare inherited disorder patients accumulate large quantities of phytanic acid?

Refsum's disease Signup and view all the answers

Which dietary restriction can ameliorate the symptoms of Refsum's disease?

Limit ruminant meat consumption Signup and view all the answers

What is the enzyme responsible for exchanging CoA for carnitine during the transport of fatty acids into the mitochondria?

Carnitinepalmitoyltransferase I Signup and view all the answers

What is the product of Acyl-CoA dehydrogenation during the β-oxidation of fatty acids?

Trans Δ 2- enoyl-CoA Signup and view all the answers

What catalyzes the hydration of the double bond to form L-3hydroxyacyl CoA in the β-oxidation process?

Enoyl CoA hydratase Signup and view all the answers

Which enzyme is involved in the 2nd dehydrogenation step of β-oxidation, leading to the formation of a ketone?

βhydroxyacyl CoA dehydrogenase Signup and view all the answers

What type of reaction is catalyzed by β-ketoacyl CoA thiolase during the formation of CoA in the β-oxidation pathway?

Thiolysis Signup and view all the answers

What are the final products of β-oxidation of fatty acids?

Acyl CoA and a long chain fatty acyl CoA Signup and view all the answers