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UNE Medical Biochemistry Unit 3 Midterm
Test EXAM LATEST VERSION 2026
QUESTIONS
1. A patient with fasting hypoglycemia, lactic acidosis, and
hepatomegaly is found to have a deficiency in glucose-6-
phosphatase. Which condition is most likely?
A. Von Gierke disease
B. McArdle disease
C. Pompe disease
D. Cori disease
Answer: A
Rationale: Glucose-6-phosphatase deficiency (type I glycogen
storage disease) prevents final release of free glucose from
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G6P, causing fasting hypoglycemia, lactic acidosis, and
glycogen accumulation in liver/kidneys.
2. During intense exercise, skeletal muscle produces lactate
that is converted back to glucose in the liver. This process is
called:
A. Gluconeogenesis
B. Glycogenesis
C. Cori cycle
D. Krebs cycle
Answer: C
Rationale: The Cori cycle shuttles lactate from muscle to liver
for gluconeogenesis; the glucose returns to muscle. Key for
maintaining blood glucose during exertion.
3. A 2-year-old presents with hepatomegaly, seizures during
fasting, and normal lactate. Liver biopsy shows normal G6P
but defective debranching enzyme. Diagnosis?
A. Von Gierke
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B. Cori disease (type III)
C. McArdle (type V)
D. Hers disease (type VI)
Answer: B
Rationale: Cori disease (debrancher enzyme deficiency) causes
hypoglycemia + hepatomegaly but normal lactate (unlike type
I). Glycogen structure is abnormal with short outer chains.
4. Which enzyme is deficient in pyruvate dehydrogenase
(PDH) deficiency?
A. Pyruvate carboxylase
B. E1 (pyruvate decarboxylase)
C. Lactate dehydrogenase
D. Malate dehydrogenase
Answer: B
Rationale: PDH complex has E1 (thiamine pyrophosphate–
dependent), E2, E3. E1 deficiency → lactic acidosis,
neurological dysfunction, and increased alanine.
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5. A marathon runner collapses after 25 miles. Blood glucose is
45 mg/dL. Which hormone would be MOST elevated in
response?
A. Insulin
B. Glucagon
C. Aldosterone
D. ANP
Answer: B
Rationale: Hypoglycemia triggers glucagon release from α-
cells of pancreas, promoting glycogenolysis and
gluconeogenesis. Insulin would be suppressed.
6. In the liver, the first committed step of gluconeogenesis is:
A. Pyruvate → Oxaloacetate
B. Oxaloacetate → Phosphoenolpyruvate
C. Fructose-1,6-bisphosphate → Fructose-6-phosphate
D. Glucose-6-phosphate → Glucose
Answer: A
UNE Medical Biochemistry Unit 3 Midterm
Test EXAM LATEST VERSION 2026
QUESTIONS
1. A patient with fasting hypoglycemia, lactic acidosis, and
hepatomegaly is found to have a deficiency in glucose-6-
phosphatase. Which condition is most likely?
A. Von Gierke disease
B. McArdle disease
C. Pompe disease
D. Cori disease
Answer: A
Rationale: Glucose-6-phosphatase deficiency (type I glycogen
storage disease) prevents final release of free glucose from
,Page 2 of 167
G6P, causing fasting hypoglycemia, lactic acidosis, and
glycogen accumulation in liver/kidneys.
2. During intense exercise, skeletal muscle produces lactate
that is converted back to glucose in the liver. This process is
called:
A. Gluconeogenesis
B. Glycogenesis
C. Cori cycle
D. Krebs cycle
Answer: C
Rationale: The Cori cycle shuttles lactate from muscle to liver
for gluconeogenesis; the glucose returns to muscle. Key for
maintaining blood glucose during exertion.
3. A 2-year-old presents with hepatomegaly, seizures during
fasting, and normal lactate. Liver biopsy shows normal G6P
but defective debranching enzyme. Diagnosis?
A. Von Gierke
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B. Cori disease (type III)
C. McArdle (type V)
D. Hers disease (type VI)
Answer: B
Rationale: Cori disease (debrancher enzyme deficiency) causes
hypoglycemia + hepatomegaly but normal lactate (unlike type
I). Glycogen structure is abnormal with short outer chains.
4. Which enzyme is deficient in pyruvate dehydrogenase
(PDH) deficiency?
A. Pyruvate carboxylase
B. E1 (pyruvate decarboxylase)
C. Lactate dehydrogenase
D. Malate dehydrogenase
Answer: B
Rationale: PDH complex has E1 (thiamine pyrophosphate–
dependent), E2, E3. E1 deficiency → lactic acidosis,
neurological dysfunction, and increased alanine.
, Page 4 of 167
5. A marathon runner collapses after 25 miles. Blood glucose is
45 mg/dL. Which hormone would be MOST elevated in
response?
A. Insulin
B. Glucagon
C. Aldosterone
D. ANP
Answer: B
Rationale: Hypoglycemia triggers glucagon release from α-
cells of pancreas, promoting glycogenolysis and
gluconeogenesis. Insulin would be suppressed.
6. In the liver, the first committed step of gluconeogenesis is:
A. Pyruvate → Oxaloacetate
B. Oxaloacetate → Phosphoenolpyruvate
C. Fructose-1,6-bisphosphate → Fructose-6-phosphate
D. Glucose-6-phosphate → Glucose
Answer: A
