Products accumulated in Hunter’s syndrome | PGI May 2017 Preparations MCQ

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