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Heme Synthesis and Disorders Quiz

Total questions: 20

Worksheet time: 10mins

Name
Class
Date
1.

Which amino acid is the precursor for heme synthesis?

a)

Glycine

b)

Alanine

c)

Glutamate

d)

Lysine

2.

Which enzyme catalyzes the first step of heme synthesis?

a)

ALA synthase

b)

ALA dehydratase

c)

Ferrochelatase

d)

Porphobilinogen deaminase

3.

Which coenzyme is required for ALA synthase?

a)

PLP

b)

FAD

c)

THF

d)

CoA

4.

Porphobilinogen is formed from which intermediate?

a)

ALA

b)

Uroporphyrinogen I

c)

Protoporphyrin IX

d)

Hematin

5.

Which is the rate-limiting enzyme of heme synthesis?

a)

ALA synthase

b)

Ferrochelatase

c)

Uroporphyrinogen decarboxylase

d)

Hem oxygenase

6.

Which metal ion is inserted into protoporphyrin to form heme?

a)

Iron

b)

Zinc

c)

Copper

d)

Magnesium

7.

Which enzyme catalyzes the insertion of iron into protoporphyrin IX?

a)

Ferrochelatase

b)

ALA synthase

c)

Uroporphyrinogen synthase

d)

Porphobilinogen deaminase

8.

Acute intermittent porphyria is due to deficiency of which enzyme?

a)

Porphobilinogen deaminase

b)

Ferrochelatase

c)

ALA synthase

d)

Uroporphyrinogen decarboxylase

9.

Which porphyria leads to photosensitivity?

a)

Porphyria cutanea tarda

b)

Acute intermittent porphyria

c)

ALA dehydratase deficiency

d)

Lead poisoning

10.

In lead poisoning, which enzymes are inhibited?

a)

ALA dehydratase and Ferrochelatase

b)

ALA synthase and Hem oxygenase

c)

Porphobilinogen deaminase and Uroporphyrinogen decarboxylase

d)

Ferrochelatase and Hem oxygenase

11.

Which form of bilirubin is water soluble?

a)

Conjugated bilirubin

b)

Unconjugated bilirubin

c)

Delta bilirubin

d)

Free bilirubin

12.

Which enzyme conjugates bilirubin in the liver?

a)

UDP-glucuronyl transferase

b)

Hem oxygenase

c)

Ferrochelatase

d)

ALA synthase

13.

Unconjugated hyperbilirubinemia is seen in?

a)

Crigler-Najjar syndrome

b)

Dubin-Johnson syndrome

c)

Obstructive jaundice

d)

All of the above

14.

Which type of bilirubin increases in obstructive jaundice?

a)

Conjugated bilirubin

b)

Unconjugated bilirubin

c)

Delta bilirubin

d)

Total bilirubin

15.

Sickle cell anemia is due to substitution of which amino acid?

a)

Valine replaces Glutamic acid

b)

Lysine replaces Glutamic acid

c)

Glycine replaces Valine

d)

Alanine replaces Lysine

16.

Thalassemia is a disorder of?

a)

Globin chain synthesis

b)

Heme synthesis

c)

Iron metabolism

d)

Bilirubin conjugation

17.

HbF consists of which chains?

a)

α2γ2

b)

α2β2

c)

α2δ2

d)

β2γ2

18.

Which hemoglobin has highest affinity for oxygen?

a)

HbF

b)

HbA

c)

HbA2

d)

Methemoglobin

19.

Which is the normal adult hemoglobin?

a)

HbA

b)

HbF

c)

HbA2

d)

HbS

20.

Which test is used for detection of Sickle Cell Trait?

a)

Sickling test

b)

Van den Bergh test

c)

Schilling test

d)

Sudan test