Reaction: IDUA hydrolyses Heparan sulfate chain(6)
- in pathway: HS-GAG degradation
An L-iduronic acid residue can be cleaved from either heparan sulfate or dermatan sulfate by the lysosomal enzyme alpha-L-iduronidase (IDUA) (Scott et al. 1991). Defects in IDUA are the cause of mucopolysaccharidosis type IH (MPS IH, Hurler syndrome, MIM:607014), mucopolysaccharidosis IH/S (MPSIH/S, HurlerScheie syndrome, MIM:607015) and mucopolysaccharidosis type IS (MPSIS, Scheie syndrome, MIM:607016) (LeeChen et al. 1999).
Reaction - small molecule participants:
Heparan sulfate chain(7) [lysosomal lumen]
aldehydo-L-iduronic acid [lysosomal lumen]
H2O [lysosomal lumen]
Heparan sulfate chain(6) [lysosomal lumen]
Reactome.org reaction link: R-HSA-2090037
======
Reaction input - small molecules:
water
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
Reaction output - small molecules:
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
aldehydo-L-iduronate
Reactome.org link: R-HSA-2090037