HOLIDAY QUIZ….

MD1

1. Biotin is a cofactor in the transfer of one carbon units at which oxidation level?
A. -CH3
B. -CH2-
C. =CH-
D. -CH=O
E. -CO2

2. Adaptation to high altitudes results in an increase in the concentration of 2,3-

diphosphoglycerate (DPG) from ~5 mM to ~8 mM inisde the red blood cells. What effect
will the higher level of DPG have on the O2 binding affinity of hemoglobin in the red
blood cells?
A. Both P50 and O2 binding affinity decrease.
B. Both P50 and O2 binding affinity increase.
C. P50 decreases and O2 binding affinity increases.
D. P50 increases and O2 binding affinity decreases.
E. Both P50 and O2 binding affinity remain the same.

3. A patient with obstructive lung disease has adapted to the chronic hypoxia. Using the
appropriate oxygen saturation curve shown below, determine the P50 value for
hemoglobin binding of oxygen in this person’s erythrocytes.

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This value is closest to:
A. 15 mm Hg
B. 20 mm Hg
C. 25 mm Hg
D. 40 mm Hg
E. 100 mm Hg

4. von Gierke's disease is characterized by a deficiency in glucose-6-phosphatase. One

complication of von Gierke's disease is an increased risk of gout. This complication is
primarily due to increased intracellular concentration of glucose-6-phosphate and
increased production of nucleotides. What metabolic pathway can use glucose-6-
phosphate to drive the overproduction of nucleotides?
A. Gluconeogenesis
B. Glycolysis
C. Pentose phosphate pathway
D. TCA cycle
E. Electron transport chain

5. What are the enzymes that catalyze the three regulated steps of glycolysis?
A. glyceraldehyde-3-phosphate dehydrogenase, phosphofructokinase, pyruvate kinase
B. hexokinase, phosphofructokinase, pyruvate kinase
C. fructose-1,6-bisphosphatase, hexokinase, phosphofructokinase
D. hexokinase, glyceraldehyde-3-phosphate dehydrogenase, phosphofructokinase
E. phosphoglycerate kinase, hexokinase, phosphofructokinase

6. The production of heat in brown adipose tissue is carried out by the thermogenin
protein. Thermogenin produces heat by what mechanism?

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A. uncoupling of the electron transport chain from ATP synthase (complex V)
B. Storing excess NADH to drive the electron transport chain
C. Increasing the activity of Complex I of the electron transport chain
D. Increasing the membrane potential of the inner mitochondrial membrane
E. Promoting the use of fatty acid oxidation in a well fed state

7. Which of the following correctly describes the reaction of the TCA cycle that is
catalyzed by citrate synthase?
A. Regulated by oxaloacaetate
B. Produces a precursor for fatty acid synthesis
C. Regulated by succinyl-CoA
D. A and B
E. All of the above

8. Pompe's disease affects all organs and is characterized by a deficiency in -1,4-

glucosidase. In a patient with Pompe's disease, which of the following would best
describe their glycogen stores?
A. decreased amount, normal branches
B. increased amount, excessive branching
C. decreased amount, absence of branching
D. increased amount, absence of branching
E. increased amount, normal branching

9. Thiamine deficiency can lead to Beri-Beri and Wenicke-Korsakoff syndrome. Which

of the following lists enzymes that are directly affected by a thiamine deficiency?
A. pyruvate dehydrogenase, isocitrate dehydrogenase, transaldolase
B. -ketoglutarate dehydrogenase, pyruvate dehydrogenase, transketolase
C. isocitrate dehydrogenase, -ketoglutarate dehydrogenase, pyruvate dehydrogenase
D. transaldolase, transketolase, pyruvate dehydrogenase
E. malate dehydrogenase, -ketoglutarate dehydrogenase, pyruvate dehydrogenase

10. Which of the following best describes phosphoenolpyruvate carboxykinase

(PEPCK)?
A. catalyzes the conversion of oxaloacetate to phosphoenolpyruvate
B. is located in the cytosol
C. is negatively regulated by glucagon
D. A and B
E. all of the above

11. The reciprocal regulation of glycogen synthesis and breakdown is primarily

controlled by which of the following?
A. hormonal regulation of adenylate cyclase and biological amplification
B. glucagon mediated inhibition of phosphorylase kinase and subsequent activation of
glycogen synthase
C. cAMP mediated regulation of kinases that lead to the activation of glycogen
phosphorylase and glycogen synthase

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D. A and C
E. All of the above

12. A patient is suffering from type 1 diabetes. She takes an overdose of insulin, resulting
in which of the following effects in the liver?
A. Increased activity of glycogen phosphorylase.
B. Increased activity of fructose-2,6-bis phosphatase.
C. Increased activity of pyruvate dehydrogenase.
D. Decreased activity of fatty acyl transferase.
E. Decreased activity of HMG CoA reductase.

13. Epinephrine acts on both muscle and liver and alters metabolism by:
A. inhibiting glycolysis in both
B. stimulating glycolysis in both
C. inhibiting glycolysis in muscle; stimulating it in liver
D. stimulating glycolysis in muscle; inhibiting it in liver

14. Glucose-6-phosphate is a key intermediate in metabolism. It is a direct substrate or

product of all the following enzymes in liver EXCEPT
A. glycogen synthase
B. glucose-6-P dehydrogenase
C. phosphoglucose isomerase
D. hexokinase
E. glucokinase

15. A patient suffers from a genetic defect that causes her fructose-2,6,-bisPhosphatase
enzyme to remain activated in a well fed state. Which of the following ENZYMEs would
be aberrantly ACTIVATED in this patient?
A. Phosphofructokinase (PFK1)
B. Glyceraldehyde 3-P dehydrogenase
C. Phosphoglucose isomerase
D. Fructose 1,6-bisPhosphatase (FBP1)
E. Pyruvate Kinase

16. A person has consumed a large bag of candy corn and his blood glucose is very high.
Which of the following best describes the activity of hexokinase and glucokinase in the
liver?

Hexokinase Glucokinase
A. At Vmax At Vmax
B. Below Vmax Below Vmax
C. At Vmax Below Vmax

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D. Below Vmax At Vmax

17. A person suffering from a genetic defect that drastically decreases the activity of
fructose 1-phosphate aldolase (fructose-1-P aldolase) is at risk of liver damage following
a diet high in fructose. What is the best explanation for this complication?
A. A build up of glyceraldehyde
B. ATP depletion due to fructose 1-P build up (i.e. Pi sequestration)
C. high, unregulated fructokinase activity
D. B and C
E. All of the above

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18. A patient who happens to be a body builder comes into your office to ask questions
concerning her new diet regime that consists of a daily intake of 25-30 raw eggs. You
realize that this patient is at risk of a biotin deficiency. What gluconeogenic enzyme
would be affected by the loss of this cofactor?
A. phosphoenol pyruvate carboxykinase
B. pyruvate carboxylase
C. glucose-6-phosphatase
D. fructose 1,6-bisphosphatase
E. pyruvate kinase

19. Following the consumption of a high carbohydrate meal, which of the following
groups of enzymes would you expect to be NEGATIVELY regulated?
A. phosphoenolpyruvate carboxykinase, glucose 6-phosphatase, fructose 1,6-
bisphosphatase
B. fructose 1,6-bisphosphatase, phosphofructokinase, hexokinase
C. phosphoenolpyruvate carboxykinase, pyruvate kinase, pyruvate carboxylase
D. glucose 6-phosphatase, glyceraldehyde 3-P dehydrogenase, glucokinase
E. phosphofructokinase, phosphoenolpyruvate carboxykinase, pyruvate kinase

20. Following the release of Acetyl CoA from the pyruvate dehydrogenase complex,
what two cofactors are necessary for the regeneration of lipoic acid?
A. thiamine pyrophosphate and NAD
B. NAD and biotin
C. Biotin and FAD
D. FAD and NAD
E. thiamine pyrophosphate and FAD

21. The pyruvate dehydrogenase (PDH) complex is tightly regulated by hormones and
cellular metabolites. Which of the following are responsible for ACTIVATING the
kinase involved in lowering the activity of the PDH complex?
A. Acetyl CoA and Insulin
B. ADP and glucagon
C. Acetyl CoA and pyruvate
D. NADH and Insulin
E. Acetyl CoA and NADH

22. Which of the following correctly describes the TCA cycle?

A. Makes twice as much NADH from one mole glucose than from one mole of alanine
B. Produces one molecule of ATP per cycle
C. Is activated by high levels of NADH and FADH2 within the cell
D. A and B
E. All of the above

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23. A patient with a defective malate dehydrogenase enzyme has unnaturally high
intracellular levels of oxaloacetate. The increase level of oxaloacetate would most
directly lead to the increased activity of which TCA cycle enzyme?
A. fumarase
B. succinate thiokinase
C. succinate dehydrogenase
D. aconitase
E. citrate synthase

24. A farmer is brought into the emergency room after an incident near a manure pit
where he collapsed. He displays no indication of heart problems and you deduce he has
been exposed to hydrogen sulfide. If you could isolate his mitochondria and test them for
oxidation state of the cofactors and the ability to consume oxygen, which of the following
would best describe the profile of cofactors and the effect of an additional treatment of
2,4-DNP to the isolated poisoned mitochondria?

Cofactors Effect of 2,4-DNP co-treatment

A. reduced cytochrome c oxygen consumption restored
B. oxidized cytochrome c oxygen consumption is inhibited
C. reduced cytochrome c oxygen consumption is inhibited
D. oxidized Coenzyme Q10 oxygen consumption is inhibited
E. reduced Coenzyme Q10 oxygen consumption is restored

25. Complex V (aka ATP synthase) is the enzyme that produces 95% of your ATP.
Which of the following is NOT true about complex V?
A. It requires a membrane potential across the inner membrane of the mitochondria to
function.
B. It uses NAD as an essential cofactor.
C. Oligomycin inhibits its function by blocking flow of protons through the complex.
D. ATP production is dependent upon the presence of ADP.
E. It can generate more ATP per mole of NADH then it can per mole of FADH2, when
oxidized by the electron transport chain.

26. Coenzyme Q is correctly described by all of the following EXCEPT?

A. can carry protons and electrons
B. can be reduced by Complex I and Complex II
C. reduced CoQ10 (aka ubiquinol) transfers its electron to Complex III
D. Accepts reducing equivalents from cytochrome a
E. Is lipid soluble

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27. A very rapidly growing tumor requires much more ribose -5-P than NADPH from the
pentose phosphate pathway. What two enzymes are required to produce ribose-5-P from
glycolytic intermediates without producing any NADPH?
A. lactonase and glucose-6-P dehydrogenase
B. glucose -6-P dehydrogenase and ribulose-5-P isomerase
C. transaldolase and transketolase
D. transketolase and ribulose-5-P isomerase
E. lactonase and transaldolase

28. Which of the following is NOT true of Glucose -6-P dehydrogenase?

A. Is the first committed step in the pentose phosphate pathway.
B. Deficiencies can lead to hemolytic anemia under conditions of oxidative stress.
C. Is regulated by ATP.
D. Catalyzes the production of 6-phosphogluconolactone from glucose-6-P.
E. Is essentially irreversible.

29. Which of the following best describes glycogen synthase?

A. Can use a single glucose molecule as a starting point
B. Is responsible for the creation of 1,4 and 1,6 glycosidic bonds
C. It is active when phosphorylated
D. Requires ATP to recycle the UDP that is produced
E. Glucagon can activate it

30. Glycogen phosphorylase is positively regulated by which group of compounds?

A. epinephrine, ATP and glucose
B. glucagon, glucose-1-P and AMP
C. glucagon, epinephrine and ATP
D. ATP, glucose and glucose-6-P
E. Epinephrine, AMP and glucagons

31. Gluconeogenesis requires all of the following enzymes to get passed the "essentially
irreversible" steps of glycolysis EXCEPT?
A. Pyruvate carboxylase
B. Glucose 6-phosphatase
C. Malate dehydrogenase
D. Fructose 1,6-bisphosphatase (FBP1)
E. Phosphoglycerate phosphatase

32. Which of the following describe the -ketoglutarate dehydrogenase complex?

A. Release one mole of CO2 per mole of -ketoglutarate oxidized
B. Negatively regulated by succinyl CoA
C. Produces one reducing equivalent and a building block for heme biosynthesis
D. A and B
E. All of the above

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33. In glycolysis, transfer of reducing equivalents occurs between which two
enzymes?

A. glyceraldehyde 3-phosphate dehydrogenase and lactate dehydrogenase

B. pyruvate kinase and glyceraldehyde 3-phosphate dehydrogenase
C. phosphofructokinase and triose-P-isomerase
D. hexokinase and pyruvate kinase
E. phosphoglycerate kinase and pyruvate kinase

34. In the case study discussed in class, the small boy with a genetic defect in the
pyruvate dehydrogenase (PDH) complex was helped with oral lipoic acid. What
enzyme in the PDH complex
is lipoic acid directly attached to?

A. Pyruvate decarboxylase
B. Dihydrolipoyl transacetylase
C. Dihydrolipoyl dehydrogenase
D. Pyruvate acetyltransferase

35. The pyruvate d ehydrogenase (PDH) complex is controlled by a variety of direct

and indirect mechanisms. How do pyruvate and NAD+ regulate the PDH complex?
A. Directly, by inhibiting the PDH complex
B. Indirectly, by activating the phosphatase
C. Indirectly, by activating the kinase
D. Indirectly, by inhibiting the kinase
E. Indirectly, by inhibiting the phosphatase

36. Comple x III of the ele ctron transport chai n produces reduced cytochrome c.
What does this complex use as a substrate?

A. NADH
B. Succinate
C. Oxidized cytochrome b
D. Reduced coenzyme Q10

37. Thermogenin plays a role in the production of heat, especially in infants.

Thermogenin produces heat by what mechanism?
A. Production of ATP
B. Uncoupling of mitochondria
C. Production of reducing equivalents
D. Maintenance of proton gradient
E. Anaplerotic processes

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38. Classical galactosemia is caused by a deficiency in what enzyme?
A. Galactokinase
B. Lactase
C. Galactose 1-phosphate uridyltransferase
D. Lactose synthase
E. Phosphoglucomutase

39. What enzyme of the glycolytic pathway uses substrate level phosphorylation to
produce a high-energy product?
A. Phosphofructokinase
B. Hexokinase
C. Phosphoglycerate mutase
D. Glyceraldehyde -3-P-dehydrogenase
E. enolase

40. In one mode of operation of the PPP, ribose-5-P can be produced without
producing NADPH. Which of the following pairs of enzymes would both be used in
that mode?
A. transketolase and transaldolase
B. glucose-6-P dehydrogenase and lactonase
C. transketolase and glucose-6-P dehydrogenase
D. transaldolase and lactonase

41. Why is glycogen stored in the liver so important to the function of the brain
(maintaining blood glucose), while that stored in muscle is not?
A. Glycogen stores in the muscle cannot be as rapidly broken down.
B. Glycogen stores in the liver are larger than those in muscle.
C. Glycogen in the liver is converted directly to glucose, while that in the muscle goes
first to glu-1-P then to glu-6-P and then to glucose
D. The muscle lacks the enzyme glucose-6-phosphatase.

42. The process of gluconeogenesis is inhibited by low energy levels in the cell.
Which of the following are important in signalling this condition to key enzymes in
the pathway?
A. low blood glucose
B. AMP
C. fructose-2, 6-bis P
D. citrate
E. glucagon

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43. The branches in the glycogen polymer are
A. made by introducing "-1,4-glycosidic linkages into a linear " 1,6 linked chain.
B. important for making glycogen more rapidly mobilized and stored.
C. very rare (every 100 glucose units).
D. only present in the liver form of glycogen.

44. A patient is brought into the emergency room near death. You have determined
that he has been exposed to a mitochondrial poison. His life depends on determining
to what poison he has been exposed. You quickly determine that his mitochondria are
not making ATP nor utilizing O2. Addition of 2,4 DNP does not restore oxygen
consumption and he has a build up of reduced Coenzyme Q (QH2) with little reduced
cytochrome c. What poison caused the patient’s condition?

A. Amytal
B. Hydrogen Sulfide
C. Carbon monoxide
D. Antimycin
E. Oligomycin

45. Which of the following CORRECTLY describes the pentose phosphate pathway
A. Produces NADPH during the non-oxidative phase
B. glucose-6-phosphate dehydrogenase catalyzes the committed step
C. Produces 4 moles of NADPH per mole of glucose-6-phosphate converted to
pentose-5- phosphate
D. Cannot produce ribose-5-phosphate without producing NADPH

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46. A patient with a genetic defect in glucose-6-phosphate dehydrogenase wants to
take a trip to Africa and is required to take mefloquine (an antimalarial drug) prior to
obtaining her visa. Two nights after starting her mefloquine, she has a special meal of
fava beans and liver. Soon after finishing the meal she collapses and is rushed into
the emergency room where she is diagnosed with severe hemolytic anemia. What of
the following best describes a likely cause of her anemia?
A. The mefloquine is inhibiting her electron transport chain and she has run out of
ATP
B. inability to maintain NADPH within her RBCs following the oxidative stress of
mefloquine and fava beans
C. inability to maintain a pool of reduced glutathione because of the genetic defect
and high oxidative power of the mefloquine and fava beans
D. B and C
E. None of the above

47. Which of the following INCORRECTLY describes the synthesis of glycogen?

A. requires a ‘primer’ glycogen molecule
B. Glycogen synthase catalyzes the formation of a 1,6 glycosidic bond
C. Requires ATP to maintain proper UTP levels
D. A pyrophosphatase drives the synthesis of UDP glucose by removing the product, PPi

48. The reciprocal regulation between glycogen synthase and glycogen phosphorylase
involves which of the following?
A. Biological amplification
B. cAMP production
C. protein kinases
D. A and C
E. all of the above

49. Which of the following best describes the glycogen stores of a patient with Von
Gierke=s disease (gluose 6-phosphatase deficiency)?

A.mount Branching
A. Higher than normal Normal branching
B. Normal Short outer branches
C. Lower than normal Normal branching
D.Higher than normal Very long branches
E.Higher than normal Short outer branches

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50. During a drug screening program, you find a chemical compound that decreases
the activity of the enzyme monoamine oxidase. A fixed dose of the drug reduces the
catalytic activity of the enzyme by the same percentage at all substrate concentrations,
with a decrease in Vmax. Km is unaffected. Which of the following best describes
this drug?

A. allosteric positive effector

B. competitive inhibitor
C. feedback inhibitor
D. noncompetitive inhibitor
E. substrate activator

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