1)Release of amino acid nitrogen and important step generating precursors for the urea cycle.

Which
statement best describes this release of amino acid nitrogen as NH from amino acids ?

Glutamato deamination by oxidation is readily reversible .

2) Proton entry into the mitochondrial matrix drives

synthase the synthesis of ATP via the ATP

3) Three reaction sequences of gluconeogenesis differ from the corresponding steps of glycolysis. They
involve ?

the conversion of pyruvate to phosphoenolpyruvate (PEP) and the reactions that remove phosphate
from fructose 1,6-bisphosphate to form fructose 6-phosphate and from glucose 6-phosphate to form
glucose

4) Consider a person with type 1 diabetes who has neglected to take insulin for the past 72 hours and
also has not eaten much. Which one of the following best describes the activity level of hepatic enzymes
involved in glycogen metabolism under these conditions?

Glycogen synthase: Inactive

Phosphorylase kinase: Active

Glycogen phosphorylase: Active

5) The red blood cells require ATP In order to maintain gradients across the the absence of these
gradients, the red blood , bringing about hemolytic anemia. Red their energy which one of the
following?

Substrate level phosphorylation

6) Succinate dehydrogenase differs from all other enzymes in the TCA cycle in that it is the only enzyme
that displays which one of the following characteristics?

It is embedded in the inner

7) An individual lactic acidemia well as reduced activity of a- dehydrogenase activity. The most mutation
that led would be in which one of the following proteins ?

The E3 subunit of pyruvate dehydrogenase

8) A distance runner is training for her half marathon and as part of the training is allowing her muscles
to use fatty acids as a fuel source. Fatty acids are converted to acetyl-CoA in the mitochondria, at which
point the acetyl-CoA can be axidized in the TCA cycle to generate reduced cofactors. Which one of the
follawing correctly describes how the acetyl-CoA is metabolized in the mitochondria?

All of the energy for high-energy phosphate bonds is derived from oxidative phosphorytation
9) without steady supply of glucose to the bloodstream, patient would become and, blood glucose levels
get low enough, experience or even a comeWhich the following necessary for the maintenance of
normal blood glucose?

Liver glucose 6-P

10) Fructose 2,6-bisphosphate is an inhibitor of ?

A fructose -bisphosphatase

11) Which one of the following statements about the urea cycle is correct?

Urinary is increased by a diet rich in protein

12) Each cytochrome is a protein that contains a bound ?

Heme

13) The fatty acidis activated by ?

acyl CoA synthetase;

14) Every human cell has the capacity to use glycolysis for energy production. Which one of the
following statements correctly describes an aspect of glycolysis?

Two molecules of ATP are used in the beginning of the pathway

15) Glycogen is the storage form of glucose and its synthesis and degradation is carefully regulated.
Which one statement below correctly describes glycogen synthesis and/or degradation

glycogen is both formed and degrades to glucose-1-P

16) A muscle biopsy specimen from a patient with a rare disorder, Luft disease, showed abnormally
large mitochondria that contained packed cristae when examined in the electron microscope. Basal
ATPase activity of the mitochondria was seven times greater than normal. From these and other data it
was concluded that oxidation and phosphorylation were partially uncoupled. Which of the following
statements about this patient is correct?

The patient shows hypermetabolism and elevated core temperature.

17) Under conditions of rapid amino acid degradation, ammonia levels increase and must be safely
removed from the cells. Glutamate can accept a nitrogen in the form of ammonia to form glutamine.
Which statement correctly describes what happens to the glutamine, and resulting ammonia and
glutamate via the action of glutaminase, in various tissues?

In the intestine, glutamine is used as fuel

18) The major precursors for glucose production are ?

lactate, amino acids, and glycerol.

19) Cytochrome transfers electrons from the b - c_{1} complex to ?

Cytochrome c oxidase

20) A newborn child has been determined to be unable to oxidize phytanic acid and is placed on a diet
containing very low levels of this unusual fatty acid . The organelle most likely to contain the defective ,
or be altered in this disorder , which one of the following ?

Peroxisome

21) The reaction catalyzed by phosphotructokinase-t ?

is the rate-limiting reaction of the glycolytic pathway.

22) Which one at the following conditions the oxidation of acetyl citric acid cycle?

.A low NAD+/NADH ratio due

23) Glycolysis generates energy such that cells have a source of energy to survive. Starting with
glyceraldehyde 3-P and synthesizing one molecule of pyruvate, the net yield of ATP and NADH would be
which one of the following?

2 ATP, 1 NADH

24) The B-Oxidation Pathway: 2nd step - An OH from water is added to the B-carbonand and an H from
water added to the a-carbon. The enzyme for this reaction is called an :

enoyl hydratase

25) Phosphofructokinase 1is inhibited by

AMP

26) A muscle biopsy specimen form a patient with a rare disorder, Luft disease, showed abnormally
large mitochondria that contained packed cristae when examined in the electron microscope. Basal
ATPase activity of the mitochondria was seven times greater than normal. From these and other data it
was concluded that oxidation and phosphorylation were partially uncoupled. Which of the following
statements about this patient is correct?

The patient shows hypermetabolism and elevated core temperature.

27) Which one of the following reactions is unique to gluconeogenesis?

Oxaloacetate phosphoenolpyruvate

28) A patient presented with a bacterial infection that produced an endotoxin that inhibits
phosphoenolpyruvate carboxykinase. In this patient, then, under these conditions, glucose production
from what precursors would be inhibited?

Alanine

29) Which one of the following enzymes can fix ammonia into an organic molecule?

Glutamate dehydrogenase
30) Phosphofructokinase-1 is activated by

fructose 2,6-bisphosphate

31) The synthesis of glucose from pyruvate by gluconeogenesis:

occurs exclusively in the cytosol

32) Because glucose has several metabolic routes it might take once it arrives in the cytoplasm, which
one of the following reactions would commit the glucose to following glycolytic pathway?

Fructose 6-P to fructose 1,6-bisP

33) A female neonate did well until approximately 24 hours of age when she became lethargic. A sepsis
workup proved negative. At 56 hours she started showing focal seizure activity. The plasma ammonia
level was found to be 1,100 micromol/L (normal 5-50 micromole/L). Quantitative plasma amino acid
levels revealed a marked elevation of arginosuccinate. *Which one of the following would also be
elevated in the blood of this patient?

Glutamine

34) A patient diagnosed with thiamin deficiency exhibited fatigue and muscle crampe. The muscle
cramps have been related to an accumulation of metabolic acidsWhich of the following metabolic acids
is most likely to accumulate in a thiamin deficiency

Pyruvic acid

35) In which one of the following time spans will fatty acids be the major source of fuel for the tissues of
the body?

While running the last mile of a marathon.

36) Assume that an individual had a glucagon-secreting pancreatic tumor (glucagonoma). Which of the
following is most likely to result from hyperglucagonemia?

Weight loss
37) A patient has large deposits of liver glycogen, which after an overnight fast, had shorter-than-normal
branches. This abnormality could be caused by a defective form of which one of the following proteins
or activities?

Amylo-1,6-glucosidase

38) A patient diagnosed with thiamine deficiency exhibited fatigue and muscle cramps. The muscle
cramps have been related to an accumulation of metabolic acids. Which of the following metabolic acids
is most likely to accumulate in a thiamine deficiency?

Pyruvic acid

39) Most of metabofic pathways are either anabolic (synthetic) or catabolic (degradation). Which of the
following pathways is considered as "amphibolic" in nature?

Citric acid cycle

40) A 43-year-old man presented with symptoms of weakness, tatigue, shortness of breath, and
dizziness. His hemoglobin level was less than 7 g/di inormal for a male being greater than 135 g/d. Red
blood cells isolated from the patient showed abnormally low level of lactate production, A deficiency of
which one of the following enzymes would be the most ikely cause of this patientr's anemia?

Phosphoglucose isomerase

41) cytochrome c oxidase passes electrons from cytochrome c to

Oxygen

42) Which of the following compound is a positive allosteric modifier of the enzyme pyruvate
carboxylase?

Biotin

43) A 2-year-old boy was brought into the emergency room, suffering from severe fasting hypoglycemia
.On physical examination, he was found to have hepatomegaly. Laboratory tests indicated that he also
had hyperlacticacidemia and hyperuricemia. A liver biopsy indicated that hepatocytes contained greater
than normal amounts of glycogen that was of normal structure. Enzyme assay likely confirmed a
deficiency in which of the following enzymes?

Glucose 6-phosphatase

44) In a glucose tolerance test, an individual in the basal metabolic state ingests a large amount of
glucose. If the individual is normal, this ingestion should result in which of the following?

An enhanced glycogen synthase activity in the liver

45) Which one of the following statements concerning gluconeogenesis is correct?

ARis important in maintaining blood glucose during the normal overnight fast.

46) NADH dehydrogenase doesn't contain?

several FMN and Fe-S centers

47) Elevated levels of ketone bodies can be found in the blood of people with untreated type 1 diabetes
and individuals on severe diets. A major difference in the laboratory findings of metabolites in the blood
of each type of individual (type 1 diabetes va. the die would be which of the following?

Glucose levels

48) Von Gierke's disease (TYPE a) is characterised by a deficiency of which enzyme?

the glucose-6-phosphatase (G6Pase) enzyme

49) Glucose 6-phosphate is an inhibitor of

Hexokinase
50) Under conditions of rapid amino acid degradation, ammonia levels increase and must be safely
removed from the cells. Glutamate can accept a nitrogen in the form of ammonia to form glutamine.
Which statement correctly describes what happens to the glutamine, and resulting ammonia and
glutamate via the action of glutaminase, in various tissues?

In the intestine, glutamine is used as fuel

51) The reaction catalyzed by phosphofructokinase-1:

is the rate-limiting reaction of the glycolytic pathway

52) A patient told her doctor that a friend told her that if she ate only carbohydrates and proteins and
no tats, she would no longer store fate in adipose tissue. The doctor told the patient her friend was
misinformed and then should further respond to this statement via which one of the following?

Dietary glusose is converted bry the liver into fatty acids and glycerol

53) In conversion of lactic acid to glucose, reactions of glycolytic pathway are circumvented, which the
following enzymes do not participate ?

Phosphoenolpyruvate carboxykinase

54) The urea cycle is the major mechanism in the human body for removal of nitrogen. Which statement
correctly describes steps of the urea cycle?

Citrulline is exchanged for ornithine across the mitochondrial membrane in an electroneutral exchange

55) An individual displays lactic acidemia as well as a reduced activity of a- ketoglutarate dehydrogenase
activity. The most likely single enzymatic mutation that leads to these changes would be in which one of
the following proteins?

The E3 subunit of pyruvate dehydrogenase

56) Which fatty acids must be supplied by the diet?

Linoleic acid and a-linolenic acid

57) A deficiency of which one of the following compounds would lead to an inability produce coenzyme
A?

Pantothenate

58) Under conditions of glucagon release, the degradation of liver glycogen normally produces which
one of the following?

More glucose 1-P than glucose

59) Glycogen is the body's storage form of glucose. When glycogen is degraded, glucose 1-P is formed.
Glucose 1-P can then be isomerized to glucose 6-P. Starting with glucose 1-P and ending with two
molecules of pyruvate, what is the yield of glycolysis in terms of ATP and NADH formed?

3 ATP, 2 NADH
60) Glycogen is the storage form of glucose and its synthesis and degradation is carefully regulated.
Which one statement below correctly describes glycogen synthesis and/or degradation?

glycogen is both formed and degrades to glucose-1-P

61) Under conditions of rapid amino acid degradation, ammonia levels increase and must be safely
removed from the cells. Glutamate can accept a nitrogen in the form of ammonia to form glutamine.
Which statement correctly describes what happens to the glutamine, and resulting ammonia and
glutamate via the action of glutaminase, in various tissues?

In the intestine, glutamine is used as fuel

62) A female neonate did well until approximately 24 hours of age when she became lethargic. A sepsis
workup proved negative. At 56 hours she started showing focal seizure activity. The plasma ammonia
level was found to be 1,100 micromol/L (normal 5-50 micromole/L). Quantitative plasma amino acid
levels revealed a marked elevation of arginosuccinate. *Which one of the following enzymatic activities
is most likely to be deficient in this patient?

argininosuccinate lyase

63) The reaction catalyzed by phosphofructokinase-1:

is the rate-limiting reaction of the glycolytic pathway.

64) Which one of the following sets of reactions best describes the oxidation of fatty acids?

Oxidation, hydration, oxidation, carbon-carbon bond breaking

65) McArdle's disease involves a deficiency of which enzyme?

Muscle phosphorylaso

66) Fill in the sentence: During overnight fasting, become the major fuel for cardiaoc muncle,
skeletal muscle, and liver.

Fatty acids

67) Compared with the resting state, vigorously contracting skeletal muscle show :

increase in the reduction of pyruvate to lactate

68) Which one of the following is most likaly to occur in a normal individual affter ingesting ahigh
carbohydrate meal?

Only insulin levels increase

69) If your patient has classic carnitine palmitoytransferase l deficiency, which one of the following
laboratory test results would you expect to observe?

Elevated blood acyicarnitine levels

70) Glucagon and epinephrine release lead to ?

dephosphorylation of glycogen synthase (inactivating itn and glycogen phosphorylase (activating it).
71) Which one of the following reactions is unique to gluconeogenesis ?

Oxaloacetate phosphoenolpyruvate

72) Cyanide binds to and prevents electron transport to O2

the Fe in the heme of the cytochrome c oxidase

73) A common intermediate in the conversion of glycerol and lactate to glucose is which of the
following?

Glucose 6-phosphate

74) cytochrome c oxidase passes electrons from cytochrome to ?

Oxygen

75) During exercise , stimulation of the TCA cycle results principally from which one of the following ?

Stimulation of the flux through a number of enzymes by a decreased NADH/NAD+ ratio

76) The B-Oxidation Pathway: hydroxyacyl CoA dehydrogenase transfers electrons to

NADP

77) Which TCA cycle enzyme will be directly affected with thiamine (Mitamin B1) defl clency?

a-Ketoglutarate Dehydrogenase complex

78) The following is the sum of three steps in the citric acid cycle.

A+B FAD+ H20------ C+ FADH2+ NADH

Choose the lettered answer that corresponds to the missing "A". "B", and "C" in the equation.

Reactant A Reactant B Reactant C Succinate NAD Oxaloacetate

79) Acetyl coA from the TCA Acetyl CoA from Fatty Acid FA) p-oxidation is pushed to katogenesis and
away eycle in liver because

OAA is decreased as a consequence of (1) Pyruvate Dehydrogenase DH inhibition and Pynavate

Carboxylase PC inhibition by acety COA end (2) its reduction to matate by malate dehydrogenase MO
because of the increase in the NADH/NAD. ratio by FA D-oxidation

80) Most of metabolle pathways are elther anabolic (eynthetic) or catabole (degradation. Which of the
following pathways is considered as "amphibolic" in nature?

Citric acid cycle

81) The reaction catalyzed by phosphotructokinase-1:

is the rate-limiting reaction of the glycolytic pathway

82) In contracting skeletal muscle, a sudden elevation of the cytosolic Ca2 concentration will result in:

activation of CAMP-dependent protein kinase A