1.
Identify the heteropolysaccharide from the disacc-haride repeat unit given in the picture
a. Heparan Sulphate
b. Heparin
c. Hyaluronic acid
d. Dermatan Sulphate
2. A 12-year-old intellectually disabled boy having short stature, protuberant abdomen with umbilical
hernia, prominent forehead. His vision is normal. His parents are normal. What is metabolic defect in
this disorder?
a. L-Iduronidase
b. Iduronate Sulfhatase
c. Aryl Sulfatase B
d. Beta Glucoronidase
3. Which reaction in the figure below occurs in both muscle and liver but has substantially different
qualities in the two?
a. Reaction A
b. Reaction B
c. Reaction C
d. Reaction D
4. True of isoenzymes?
a. Same rate of work, catalize same reaction and have similar kinetics
b. Same quaternary structure
c. Same group of classification used
d. Have similar distributions in organs
5. Which one of the following statements concerning glycolysis is correct?
a. Heokinase is important in hepatic glucose metabolism only in the absorptive period following
consumption of a carbohydrate-containing meal.
b. Fructose 2.6-Bisphosphate is a potent inhibitor of phosphofructokinase.
c. The regulated reactions are also the irreversible reactions.
d. The conversion of glucose to lactate yields two ATP and two NADH.
6. Enzymes involved in Gluconeogenesis:
1. Pyruvate kinase
2. PEP Carboxykinase
3. Pyruvate carboxylase
4. Glyceraldehyde 3 P dehydrogenase
Which of the following enzymes are involved in gluconeogenesis?
a. 1 and 2
b. 2and 3
c. 2, 3, 4
d. 1, 2, 3
7. A 3-year-old man presented with symptoms of weakness, fatigue, shortness of breath, and dizziness. His
hemoglobin lavel was less than 7 g dl (normal for a male being greater than 13.5 g/dl). 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 likely cause of this patient's anemia?
a. Phosphoglucose isomerase
b. Phosphofructokinase
c. Pyruvate kinase
d. glucose-6 – phosphate dehydrogenase
8. A middle aged woman presents with fissures in mouth, tingling sensation. Investigation showed
reduced glutathione reductase activity. Which vitamin deficiency causes this?
a. Vitamin B1
b. Vitamin B2
c. Vitamin B6
d. Vitamin B 12
9. A 48-year-old male with excessive drinking over an extended period of time while eating poorly,
admitted to the hospital with “high out- put” heart failure. Which of the following enzymes is most
likely inhibited?
a. Isocitrate dehydrogenase
b. α-Ketoglutarate dehydrogenase
c. Succinate thiokinase
d. Citrate synthase
10. Thiamine level is best monitored by (recent update)-
a. Transketolase level in blood
b. Thiamine level in blood
c. G6PD activity
d. Reticulocytosis
11. Identify the correct regulatory molecules:
a. A= Glucagon; B= Insulin
b. A= Epinephrine; B= Glucagon
c. A=Epinephrine ; B= Insulin
d. A= Insulin; B= Glucagon
12. A 2 year old boy presented with fasting hypoglycemia, hepatomegaly, doll like facies and thin
extremities. Kidneys are enlarged but no splenomegaly. What is the most probable GSD?
a. Type 1 GSD
b. Type II GSD
c. Type III GSD
d. Type IV GSD
13. A 3-week-old neonate who began vomiting 2 days after birth, usually within 30 minutes after
breastfeeding. He also has abdominal distension with enlargement of liver, with jaundice. The
consulting doctor did two urine dipstick test, one specific for glucose was negative, second test specific
for reducing sugar was positive. What is the diagnosis?
a. Hereditary fructose intolerance
b. Classic galactosemia
c. Essential fructosuria
d. Von gierkes disease
14. Routine examination of the urine of an asymptomatic patient showed a positive reaction with benediet
test (a copper reduction method of detecting reducing sugars). But a negative reaction with the glucose
oxidase test. Which one of the following sugars is least likely to be present (assuming a single elevated
saccharide)?
a. Lactose
b. Fructose
c. Sucrose
d. Galactose
15. Biological role of metallothionein is to sequester harmful metal ions. These bind:
a. CD++, Cu++ & Zn++
b. A1+++, Hg++ & NH4+
c. Pt+++, As+++ & PO4
d. Fe+++, Na+ & K+
16. A Buffer that is most effective at PH of about 8.3 is:
a. Acetate Buffer
b. Bicarbonate Buffer
c. Phosphate Buffer
d. Tris Buffer
17. A low level carbon dioxide labeled with C14 is accidentally released into the atmosphere surrounding
industrial workers as they resume work following the lunch hour. Unknowingly, they breathe the
contaminated air for l hour. Which of the following compounds will be radioactively labeled?
a. All of the carbon atoms of newly synthesized fatty acid
b. About one half of the carbon atoms of newly synthesized fatty acids
c. About one third of the carbons of newly synthesized malonyl CoA
d. One half of the carbon atoms of newly synthesized acetyl CoA
18. A 6-month old boy was hospitalized following a seizure. History revealed that for several days prior, his
appetite was decreased due to a "stomach virus". At admission, his blood glucose was 24 mg/dl (age-
referenced normal is 60-100). His urine was negative for ketone bodies, but positive for a variety of
dicarboxylic acids. A tentative diagnosis of medium-chain fatty acyl CoA dehydrogenase (MCAD)
deficiency is made. In patients with MCAD deficiency, the fasting hypoglycemia is a consequence of:
a. Decreased acetyl CoA production
b. Decreased ability to convert acetyl CoA to glucose
c. Increased conversion of acetyl CoA to acetoacetate
d. Increased production of ATP and NADH
19. When the liver is actively synthesizing fatty acids, a concomitant decrease in β oxidation of fatty acids is
due to which of the following?
a. Inhibition of a translocation between cellular compartments
b. Inhibition by an end product
c. Activation of an enzyme
d. Detergent in adipocyte lipolysis
20. A 4-year old boy of middle-eastern ancestry is known to have inherited favism a sensitivity to fava
beans due to glucose-6 – phosphate dehydrogenase deficiency (MIM305900). Which of the following
indicates the primary pathway affected by this deficiency and the pathway secondarily affected because
one of its substrates will be deficient?
a. Pentose phosphate pathway fatty acid synthesis
b. Pentose phosphate pathway fatty acid oxidation
c. Citric acid cycle, Pentose phosphate pathway
d. Citric acid cycle glycolysis
21. Abetalipoproteinemia is a genetic disorder characterised by malabsorption of dietry lipids steatorrhoea,
accumulation of intestinal triglcerides and hypolipoproteinemia. A deficiency of which apoprotein
would account for this clinical picture ?
a. apo B 100
b. apo B 48
c. apo CII
d. apo E
22. A 7-year old boy presented with severe abdominal pain. On examination, he had xanthomas. Blood
sample was taken for work-up, blood sample had milky appearance of plasma. Which of the following
lipoprotein is increased?
a. LDL
b. HDL
c. Chylomicron
d. Chylomicron remnants
23. On laboratory investigations in a patient, LDL was highly elevated but the level of LDL receptors was
normal. Which of the following is most probable cause?
a. Phosphorylation of LDL receptors
b. Lipoprotein lipase deficiency
c. Apo B-100 mutation
d. Cholesterol Acyl Co-A transferase deficiency
24. Niemann-Pick disease, like other neurolipidoses, present in infancy or childhood with plateauing of
development and neurologic regression. The accumulating substance is a phospholipid made in which of
the following steps in the figure below?
a. Step A
b. Step B
c. Step C
d. Step D
25. In this is figure of mitochondria location of enzymes are pointed by A, B, C. For each item listed below,
select the appropriate location from the drawing shown. Select the one best answer
a. Carnitine shuttle - B
b. F0F1 ATP synthase – C
c. HMG – CoA lyase – B
d. Carnitine acyltransferase -1 - A
26. The reaction:
Succinyl CoA + Acetoacetate →Acetoacetyl CoA + succinate occurs in all of the following EXCEPT
a. Brain
b. Striated muscle
c. Liver
d. Cardiac muscle
27. Which of the following enzymes has decreased activity in fasting except ?
a. Glucokinase
b. Hormone sensitive lipase
c. Glycogen synthase
d. Acetyl CoA carboxylase
28. In a diabetic patient
a. RQ decreases and on administrasion of insulin RQ increases
b. RQ increases and on administrasion of insulin RQ decreases
c. No effect of insulin on RQ
d. No effect of diabetes on RQ
Dr Ankur Jain
