Reaction: IDS hydrolyses Heparan sulfate chain(5)
- in pathway: HS-GAG degradation
Iduronate 2sulfatase (IDS) hydrolyses 2-sulfate groups from Liduronate 2-sulfate units of heparan sulfate. 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:
Heparan sulfate chain(6) [lysosomal lumen]
SO4(2-) [lysosomal lumen]
Heparan sulfate chain(5) [lysosomal lumen]
H2O [lysosomal lumen]
Reactome.org reaction link: R-HSA-1678650
======
Reaction input - small molecules:
beta-D-IdoA2S-(1->4)-alpha-D-GlcNS3S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
water
Reaction output - small molecules:
beta-D-IdoA-(1->4)-alpha-D-GlcNS3S-(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-1678650