UNE Medical Biochemistry Final Exam 2025 | University Of New England |
Featuring Topics such as Metabolic Pathways, Enzymatic Function,
Bioenergetics, Carbohydrate and Lipid Metabolism, and Protein Structure
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 - (CORRECT ANSWER)Hormone-sensitive lipase
β- oxidation of long-chain fatty acids is inhibited by:
Pantothenic Acid (vitamin B5)
Carnitine
Malonyl-CoA
Fatty acids - (CORRECT ANSWER)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 - (CORRECT ANSWER)FAD-
coupled oxidation, hydration, NAD+-coupled oxidation, thiolytic cleavage
, 2
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
Gluconeogenesis is inhibited by acetyl-CoA produced in the β-oxidation of fatty acids - (CORRECT
ANSWER)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 - (CORRECT ANSWER)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?
Pyridoxal phosphate
, 3
Carnitine
Thiamine
Biotin - (CORRECT ANSWER)Carnitine
An 11-year-old female is brought to the emergency department by her parent. They report she has
recently been very thirsty and needs to make frequent trips to the bathroom. Physical examination
revealed a thin girl in the 30th percentile for height and weight. A rapid dipstick test revealed glucose in
her urine. Evaluation of this girl's blood would reveal decreased levels of which of the following
compounds or hormones?
Glutamine
Ketones
Fatty acids
Insulin
Glucose - (CORRECT ANSWER)Insulin
What is the compound formed from the spontaneous decarboxylation of acetoacetate in the blood that
is responsible for a fruity odor on the breath of someone with ketonemia?
β-hydroxybutyrate
Succinyl-CoA
Acetyl-CoA
Acetone - (CORRECT ANSWER)Acetone
Fatty acid synthase is a multimeric enzyme complex. Which of the following subunits contains
pantothenic acid as a cofactor?
Featuring Topics such as Metabolic Pathways, Enzymatic Function,
Bioenergetics, Carbohydrate and Lipid Metabolism, and Protein Structure
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 - (CORRECT ANSWER)Hormone-sensitive lipase
β- oxidation of long-chain fatty acids is inhibited by:
Pantothenic Acid (vitamin B5)
Carnitine
Malonyl-CoA
Fatty acids - (CORRECT ANSWER)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 - (CORRECT ANSWER)FAD-
coupled oxidation, hydration, NAD+-coupled oxidation, thiolytic cleavage
, 2
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
Gluconeogenesis is inhibited by acetyl-CoA produced in the β-oxidation of fatty acids - (CORRECT
ANSWER)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 - (CORRECT ANSWER)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?
Pyridoxal phosphate
, 3
Carnitine
Thiamine
Biotin - (CORRECT ANSWER)Carnitine
An 11-year-old female is brought to the emergency department by her parent. They report she has
recently been very thirsty and needs to make frequent trips to the bathroom. Physical examination
revealed a thin girl in the 30th percentile for height and weight. A rapid dipstick test revealed glucose in
her urine. Evaluation of this girl's blood would reveal decreased levels of which of the following
compounds or hormones?
Glutamine
Ketones
Fatty acids
Insulin
Glucose - (CORRECT ANSWER)Insulin
What is the compound formed from the spontaneous decarboxylation of acetoacetate in the blood that
is responsible for a fruity odor on the breath of someone with ketonemia?
β-hydroxybutyrate
Succinyl-CoA
Acetyl-CoA
Acetone - (CORRECT ANSWER)Acetone
Fatty acid synthase is a multimeric enzyme complex. Which of the following subunits contains
pantothenic acid as a cofactor?
