,TABLE OF CONTENTS
BIOCHEMISTRY, 3
ANATOMY, 37
PHYSIOLOGY, 68
MICROBIOLOGY, 97
PATHOLOGY, 135
PHARMACOLOGY, 169
2
,BIOCHEMISTRY
3
, QUESTION ANSWER EXPLANATION
1. All mucopolysaccharidoses A A. Hunter syndrome
are autosomal recessive,
EXCEPT? Hunter syndrome is an X-linked recessive
disease, not autosomal recessive.
A. Hunter syndrome
B. Hurler syndrome All mucopolysaccharidoses are autosomal
C. Sanfilippo syndrome recessive except for Hunter syndrome.
D. Sly syndrome
References:
● Ferrier, D. (2017). Lippincott Illustrated Reviews:
Biochemistry (7th ed., p. 164). Lippincott Williams
and Wilkins.
● Le, T., Bhushan, V., & Sochat, M. (2023). First Aid
for the USMLE Step 1 2022, Thirty Third Edition
(33rd ed., pp. 58-59). McGraw-Hill Education.
2. Which of the following D D. Heparan sulfate
substrates accumulate in Hurler
syndrome? In Hurler syndrome, the degradation of
dermatan sulfate and heparan sulfate is
A. Sphingomyelin affected, due to a deficiency of the enzyme
B. Ceramide trihexoside α-L-iduronidase.
C. GM2 ganglioside
D. Heparan sulfate
Disease Biochemical
imbalance
Niemann-Pick Sphingomyelin
disease accumulation
Fabry disease Ceramide
trihexoside
accumulation
Tay-Sachs disease GM2 ganglioside
accumulation
References:
● Ferrier, D. (2017). Lippincott Illustrated Reviews:
Biochemistry (7th ed., p. 164). Lippincott Williams
and Wilkins.
● Le, T., Bhushan, V., & Sochat, M. (2023). First Aid
for the USMLE Step 1 2022, Thirty Third Edition
(33rd ed., p. 86). McGraw-Hill Education.
3. Hunter syndrome results D D. Iduronate-2-sulfatase
from the deficiency of which
enzyme? In Hunter syndrome, the degradation of
dermatan sulfate and heparan sulfate is
A. Galactocerebrosidase affected, due to a deficiency of the enzyme
B. Glucocerebrosidase Iduronate-2-sulfatase.
C. α-L-iduronidase
D. Iduronate-2-sulfatase
Disease Enzyme deficiency
4
BIOCHEMISTRY, 3
ANATOMY, 37
PHYSIOLOGY, 68
MICROBIOLOGY, 97
PATHOLOGY, 135
PHARMACOLOGY, 169
2
,BIOCHEMISTRY
3
, QUESTION ANSWER EXPLANATION
1. All mucopolysaccharidoses A A. Hunter syndrome
are autosomal recessive,
EXCEPT? Hunter syndrome is an X-linked recessive
disease, not autosomal recessive.
A. Hunter syndrome
B. Hurler syndrome All mucopolysaccharidoses are autosomal
C. Sanfilippo syndrome recessive except for Hunter syndrome.
D. Sly syndrome
References:
● Ferrier, D. (2017). Lippincott Illustrated Reviews:
Biochemistry (7th ed., p. 164). Lippincott Williams
and Wilkins.
● Le, T., Bhushan, V., & Sochat, M. (2023). First Aid
for the USMLE Step 1 2022, Thirty Third Edition
(33rd ed., pp. 58-59). McGraw-Hill Education.
2. Which of the following D D. Heparan sulfate
substrates accumulate in Hurler
syndrome? In Hurler syndrome, the degradation of
dermatan sulfate and heparan sulfate is
A. Sphingomyelin affected, due to a deficiency of the enzyme
B. Ceramide trihexoside α-L-iduronidase.
C. GM2 ganglioside
D. Heparan sulfate
Disease Biochemical
imbalance
Niemann-Pick Sphingomyelin
disease accumulation
Fabry disease Ceramide
trihexoside
accumulation
Tay-Sachs disease GM2 ganglioside
accumulation
References:
● Ferrier, D. (2017). Lippincott Illustrated Reviews:
Biochemistry (7th ed., p. 164). Lippincott Williams
and Wilkins.
● Le, T., Bhushan, V., & Sochat, M. (2023). First Aid
for the USMLE Step 1 2022, Thirty Third Edition
(33rd ed., p. 86). McGraw-Hill Education.
3. Hunter syndrome results D D. Iduronate-2-sulfatase
from the deficiency of which
enzyme? In Hunter syndrome, the degradation of
dermatan sulfate and heparan sulfate is
A. Galactocerebrosidase affected, due to a deficiency of the enzyme
B. Glucocerebrosidase Iduronate-2-sulfatase.
C. α-L-iduronidase
D. Iduronate-2-sulfatase
Disease Enzyme deficiency
4
