Reaction: IDS hydrolyses dermatan sulfate (Chebi:63517 chain)
- in pathway: CS/DS degradation
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).
Reaction - small molecule participants:
ChEBI:63516 chain [lysosomal lumen]
SO4(2-) [lysosomal lumen]
H2O [lysosomal lumen]
ChEBI:63517 chain [lysosomal lumen]
Reactome.org reaction link: R-HSA-1793182
======
Reaction input - small molecules:
water
beta-D-GalNAc-(1->4)-beta-D-IdoA2S-(1->3)-beta-D-GalNAc4S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
Reaction output - small molecules:
beta-D-GalNAc-(1->4)-beta-D-IdoA-(1->3)-beta-D-GalNAc4S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
sulfate
Reactome.org link: R-HSA-1793182