Page 1 of 30
UNE MEDICAL BIOCHEMISTRY ACTUAL FINAL
EXAM WITH ANSWERS
Fatty acid synthase is a multimeric enzyme complex. Which of the
following subunits contains pantothenic acid as a cofactor?
Thioesterase
Ketoacyl synthase
Acyl carrier protein (ACP)
Enoyl reductase
Acyl carrier protein (ACP)
A 55-year-old male is diagnosed with Type 2 diabetes and his
current presentation is consistent with a lack of insulin
production. Serum levels of free fatty acids were elevated in this
individual primarily due to increased activity of which of the
following enzymes?
Carnitine palmitoyl transferase I
Carbamoyl phosphate synthetase I
Hormone-sensitive lipase
Glutamate dehydrogenase
Hormone-sensitive lipase
β- oxidation of long-chain fatty acids is inhibited by:
Pantothenic Acid (vitamin B5)
Carnitine
, Page 2 of 30
Malonyl-CoA
Fatty acids
Malonyl-CoA
Which of the following represents the correct sequence of events
in a cycle of β-oxidation beginning with a saturated fatty acyl-
CoA?
NAD+-coupled oxidation, FAD-coupled oxidation, hydration,
thiolytic cleavage
NAD+-coupled oxidation, hydration, FAD-coupled oxidation,
thiolytic cleavage
hydration, FAD-coupled oxidation, NAD+-coupled oxidation,
thiolytic cleavage
FAD-coupled oxidation, hydration, NAD+-coupled oxidation,
thiolytic cleavage
FAD-coupled oxidation, NAD+-coupled oxidation, hydration,
thiolytic cleavage
FAD-coupled oxidation, hydration, NAD+-coupled oxidation, thiolytic
cleavage
Glucose cannot be synthesized from the β-oxidation of even chain
fatty acids because:
Insulin levels are elevated when fatty acids are being oxidized
The acetyl-CoA produced from β-oxidation is completely oxidized
in the TCA cycle
Acetyl-CoA produced in the β-oxidation of fatty acids cannot be
transported out of the mitochondrial matrix
, Page 3 of 30
Gluconeogenesis is inhibited by acetyl-CoA produced in the β-
oxidation of fatty acids
The acetyl-CoA produced from β-oxidation is completely oxidized in the
TCA cycle
A well, 2-year-old female presents to the clinic with symptoms of a
viral illness. Her parents report she has had bouts of vomiting and
diarrhea over the last 24 hours. This morning they could barely
wake her from her crib and she was very weak. Laboratory tests
show low blood glucose (hypoglycemia) and elevated liver
enzymes. There are no ketones present in her urine. The child is
hospitalized and glucose is administered I.V. Additional laboratory
analysis showed elevated medium-chain fatty acylcarnitines in
blood and 6-8 carbon dicarboxylic acids in the urine. Which of the
following abnormalities is the most likely diagnosis for this child?
Mitochondrial defect in fatty acid transport
Defect of medium-chain fatty acyl synthetase
Carnitine deficiency
Defect of medium-chain coenzyme A dehydrogenase
Mitochondrial defect in the electron transport chain
Defect of medium-chain coenzyme A dehydrogenase
A 1-year-old female presents to the emergency room with
hypoglycemia following a brief seizure. After laboratory tests, a
deficiency in β-oxidation is confirmed. This could potentially be
caused by a deficiency in an enzyme or reduced levels of which of
the following cofactors are required for this process?
UNE MEDICAL BIOCHEMISTRY ACTUAL FINAL
EXAM WITH ANSWERS
Fatty acid synthase is a multimeric enzyme complex. Which of the
following subunits contains pantothenic acid as a cofactor?
Thioesterase
Ketoacyl synthase
Acyl carrier protein (ACP)
Enoyl reductase
Acyl carrier protein (ACP)
A 55-year-old male is diagnosed with Type 2 diabetes and his
current presentation is consistent with a lack of insulin
production. Serum levels of free fatty acids were elevated in this
individual primarily due to increased activity of which of the
following enzymes?
Carnitine palmitoyl transferase I
Carbamoyl phosphate synthetase I
Hormone-sensitive lipase
Glutamate dehydrogenase
Hormone-sensitive lipase
β- oxidation of long-chain fatty acids is inhibited by:
Pantothenic Acid (vitamin B5)
Carnitine
, Page 2 of 30
Malonyl-CoA
Fatty acids
Malonyl-CoA
Which of the following represents the correct sequence of events
in a cycle of β-oxidation beginning with a saturated fatty acyl-
CoA?
NAD+-coupled oxidation, FAD-coupled oxidation, hydration,
thiolytic cleavage
NAD+-coupled oxidation, hydration, FAD-coupled oxidation,
thiolytic cleavage
hydration, FAD-coupled oxidation, NAD+-coupled oxidation,
thiolytic cleavage
FAD-coupled oxidation, hydration, NAD+-coupled oxidation,
thiolytic cleavage
FAD-coupled oxidation, NAD+-coupled oxidation, hydration,
thiolytic cleavage
FAD-coupled oxidation, hydration, NAD+-coupled oxidation, thiolytic
cleavage
Glucose cannot be synthesized from the β-oxidation of even chain
fatty acids because:
Insulin levels are elevated when fatty acids are being oxidized
The acetyl-CoA produced from β-oxidation is completely oxidized
in the TCA cycle
Acetyl-CoA produced in the β-oxidation of fatty acids cannot be
transported out of the mitochondrial matrix
, Page 3 of 30
Gluconeogenesis is inhibited by acetyl-CoA produced in the β-
oxidation of fatty acids
The acetyl-CoA produced from β-oxidation is completely oxidized in the
TCA cycle
A well, 2-year-old female presents to the clinic with symptoms of a
viral illness. Her parents report she has had bouts of vomiting and
diarrhea over the last 24 hours. This morning they could barely
wake her from her crib and she was very weak. Laboratory tests
show low blood glucose (hypoglycemia) and elevated liver
enzymes. There are no ketones present in her urine. The child is
hospitalized and glucose is administered I.V. Additional laboratory
analysis showed elevated medium-chain fatty acylcarnitines in
blood and 6-8 carbon dicarboxylic acids in the urine. Which of the
following abnormalities is the most likely diagnosis for this child?
Mitochondrial defect in fatty acid transport
Defect of medium-chain fatty acyl synthetase
Carnitine deficiency
Defect of medium-chain coenzyme A dehydrogenase
Mitochondrial defect in the electron transport chain
Defect of medium-chain coenzyme A dehydrogenase
A 1-year-old female presents to the emergency room with
hypoglycemia following a brief seizure. After laboratory tests, a
deficiency in β-oxidation is confirmed. This could potentially be
caused by a deficiency in an enzyme or reduced levels of which of
the following cofactors are required for this process?
