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. 2001 Nov;24(6):675-80.
doi: 10.1023/a:1012763026526.

A fluorimetric enzyme assay for the diagnosis of MPS II (Hunter disease)

Y V Voznyi  1 , J L Keulemans, O P van Diggelen
Affiliations

A fluorimetric enzyme assay for the diagnosis of MPS II (Hunter disease)

Y V Voznyi et al. J Inherit Metab Dis. 2001 Nov.

Abstract

4-Methylumbelliferyl-alpha-iduronate 2-sulphate was synthesized and shown to be a specific substrate for the lysosomal iduronate-2-sulphate sulphatase (IDS). Fibroblasts (n = 17), leukocytes (n = 3) and plasmas (n = 9) from different MPS II patients showed < 5% of mean normal IDS activity. The enzymatic liberation of the fluorochrome from 4-methylumbelliferyl-alpha-iduronate 2-sulphate requires the sequential action of IDS and alpha-iduronidase. A normal level of alpha-iduronidase activity was insufficient to complete the hydrolysis of the reaction intermediate 4-methylumbelliferyl-alpha-iduronide formed by IDS. A second incubation step in the presence of excess purified alpha-iduronidase is needed to avoid underestimation of the IDS activity.

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References

    1. Clin Chim Acta. 1990 Feb 28;187(2):131-9 - PubMed
    1. Methods Enzymol. 1979;56:527-30 - PubMed
    1. Methods Enzymol. 1978;50:439-56 - PubMed
    1. Carbohydr Res. 1979 Mar;69:203-16 - PubMed
    1. Biochem Biophys Res Commun. 1982 Sep 30;108(2):868-75 - PubMed

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