IDS hydrolyses dermatan sulfate (Chebi:63517 chain)

Stable Identifier
R-HSA-1793182
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
iduronate-2-sulfatase activity of IDS dimer [lysosomal lumen]
Physical Entity
Activity
Orthologous Events
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Created