A new route for the preparation of iduronate-2-sulfate glycosides: A new substrate for iduronate-2-sulfatase for screening and diagnosis of Mucopolysaccharidosis-II.

Gurpreet Singh, Sanghyun Lim, Joseph Wakpal, Hamid Khaledi, Michael H Gelb

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Article in Carbohydrate research, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 · Who and what money

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5 authors.

Gurpreet SinghDept. of Chemistry, University of Washington, Seattle, WA 98195, USA.

Sanghyun LimDept. of Biochemistry, University of Washington, Seattle, WA 98195, USA; Enfanos, LLC, 4000 Mason Road, #300, Seattle, WA 98195, USA.

Joseph WakpalDept. of Biochemistry, University of Washington, Seattle, WA 98195, USA; Enfanos, LLC, 4000 Mason Road, #300, Seattle, WA 98195, USA.

Hamid KhalediDept. of Biochemistry, University of Washington, Seattle, WA 98195, USA; Enfanos, LLC, 4000 Mason Road, #300, Seattle, WA 98195, USA.

Michael H GelbDept. of Chemistry, University of Washington, Seattle, WA 98195, USA; Dept. of Biochemistry, University of Washington, Seattle, WA 98195, USA; Enfanos, LLC, 4000 Mason Road, #300, Seattle, WA 98195, USA. Electronic address: gelb@uw.edu.

Funding

Multiplex Analysis of Inborn Errors of MetabolismR01DK067859 · NIDDK · UNIVERSITY OF WASHINGTON · PI GELB, MICHAEL H · 2003 to 2025

$9.8M

NIDDK NIH HHS R01 DK067859

6 · The paper itself

Abstract

Mucopolysaccharidosis type II (MPS-II) is caused by deficiency of iduronate-2-sulfatase (IDS). Newborn screening and diagnosis of MPS-II is warranted because it allows for earlier treatment leading to a better clinical outcome. Newborn screening of MPS-II is based on measurement of IDS enzymatic activity in dried blood spots using synthetic substrates. Here we describe a new synthetic route for preparation of iduronate-2-sulfate glycosides that allows for completely selective sulfation at the 2-position of the iduronate residue and avoids possible contamination by glucuronate-2-sulfate glycosides, thus avoiding any background interference from glucuronate-2-sulfatase that is also present in dried blood spots. The synthesis also allows for preparation of isotopically labeled IDS product, which is used as an internal standard in tandem mass spectrometry assays of IDS enzymatic activity.

Indexed as

GlycosidesIduronate SulfataseIduronic AcidMucopolysaccharidosis IISulfatesHumansGlycosidesIduronate SulfataseIduronic AcidSulfates

Identifiers

PMID40592241

DOI10.1016/j.carres.2025.109585

PMCPMC12326880

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