Reactome | IDS hydrolyses dermatan sulfate (Chebi:63517 chain)

IDS hydrolyses dermatan sulfate (Chebi:63517 chain)

Stable Identifier

R-HSA-1793182

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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IDS hydrolyses dermatan sulfate (Chebi:63517 chain)

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Iduronate 2-sulfatase (IDS) hydrolyses 2-sulfate groups from L-iduronate 2-sulfate units of dermatan sulfate in the lysosome. Defects in IDS are the cause of mucopolysaccharidosis type II (MPSII, MIM:309900), also called Hunter syndrome (Wilson et al. 1990).

Literature References

PubMed ID	Title	Journal	Year
2122463	Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA Wilson, PJ, Morris, CP, Anson, DS, Occhiodoro, T, Bielicki, J, Clements, PR, Hopwood, JJ	Proc Natl Acad Sci U S A	1990