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Unit Code 80945:
Iduronate Sulfatase, Fibroblasts

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Method Description

Hunter syndrome (mucopolysaccharidosis II) results from a de-

ficiency of the iduronate sulfatase enzyme. This enzyme catalyzes

the removal of sulfate groups on the C-2 position of iduronic acid

residues of heparin, heparan sulfate, and dermatan sulfate.

 

In this procedure, the tritiated disulfated disaccharide,

O-(a-L-idopyranosyluronic acid 2-sulfate)-(1-4)-2,5-anhydro-D-

[3]H-mannitol-6-sulfate, is used as the substrate. The enzyme

hydrolyses the substrate to form inorganic sulfate and labeled

monosulfated disaccharide. The monosulfated product is then

separated from the disulfated substrate by anion exchange

chromatography. (Wasteson A, Neufeld EF:  Iduronate sulfatase

from human plasma. Meth Enzymol 1982;83:573-578)

 

Performing Laboratory Location

Rochester

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