Reaction: SGSH hydrolyses Heparan sulfate chain(7)
- in pathway: HS-GAG degradation
N-sulphoglucosamine sulphohydrolase (SGSH) hydrolyses the sulfate group from the terminal N-sulphoglucosamine residue of heparan sulfate (Scott et al. 1995). Defects in SGSH cause mucopolysaccharidosis type IIIA (MPSIIIA, MIM:252900), also called Sanfilippo syndrome A (Weber et al. 1997).
Reaction - small molecule participants:
SO4(2-) [lysosomal lumen]
Heparan chain(1) [lysosomal lumen]
H2O [lysosomal lumen]
Heparan sulfate chain(7) [lysosomal lumen]
Reactome.org reaction link: R-HSA-2090043
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Reaction input - small molecules:
water
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:
sulfate
alpha-D-GlcN-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
Reactome.org link: R-HSA-2090043