Products accumulated in Hunter’s syndrome are:
A. Keratan sulfate
B. Dermatan sulfate
C. Chondroitin sulfate
D. Heparan sulfate
E. Hyaluronic acid
A. Keratan sulfate
B. Dermatan sulfate
C. Chondroitin sulfate
D. Heparan sulfate
E. Hyaluronic acid
Ans.
B. Dermatan sulfate ; (D) Heparan
sulfate
• In Hunter syndrome the accumulated
metabolites are
—Dermatan sulfate
— Heparan sulfate
• Hunter’s syndrome is X-linked recessive disorder.
• Other mucopolysaccharidoses:-
—Dermatan sulfate
— Heparan sulfate
• Hunter’s syndrome is X-linked recessive disorder.
• Other mucopolysaccharidoses:-
Type
|
Syndrome
|
Enzyme to defect
|
Accumulated Metabolite
|
I
|
Hurler or scheie
|
Iduronidase
|
Dermation sulfate Heparan sulfate
|
II
|
Hunter
|
Iduronate sulfatase
|
—
|
IIIA
|
Sandfilippo A
|
Heparan-N-sulfatase
|
Heparan sulfatase
|
IIIB
|
SandfilippoB
|
N-Acetylglucosaminidase
|
Heparan sulfatase
|
IV
|
Morquio
|
N-Acetyl galactosamine 6-sulfatase
|
Keratan sulfatase
|
VI
|
Maroteaux-Lamy
|
N-Acetyl galacto samine 4-sulfatase
|
Dermatan sulfatase
|
VII
|
Sly
|
3-Glucuronidase
|
Dermatan sulfatase; Heparan
sulfatase
|