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