Reaction: GLB1 hydrolyses a glycosaminoglycan
- in pathway: Keratan sulfate degradation
Beta-galactosidase (GLB1) can cleave terminal galactose residues from glycosaminoglycans such as keratan sulfate (KS) (Asp et al. 1969). Defects in GLB1 cause the lysosomal storage diseases GM1gangliosidosis (Yoshida et al. 1991) and Morquio syndrome type B (Oshima et al. 1991).
Reaction - small molecule participants:
Gal [lysosomal lumen]
GlcNAc(S)-Gal-GlcNAc(S)-Gal [lysosomal lumen]
H2O [lysosomal lumen]
Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Reactome.org reaction link: R-HSA-1630306
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Reaction input - small molecules:
water
beta-D-Galp-(1->4)-beta-D-GlcpNAc6S-(1->3)-beta-D-Galp-(1->4)-beta-D-GlcpNAc6S-(1->3)-D-Galp
Reaction output - small molecules:
alpha-D-galactose
beta-D-GlcpNAc6S-(1->3)-beta-D-Galp-(1->4)-beta-D-GlcpNAc6S-(1->3)-D-Galp
Reactome.org link: R-HSA-1630306