Wednesday 25 November 2015

Hunter’s syndrome | Medical PG MCQs

Products accumulated in Hunter’s syndrome are: 

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:-


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