Reaction: GLB1 hydrolyses linker chain(2)
- in pathway: HS-GAG degradation
Beta-galactosidase (GLB1) can cleave terminal galactose residues from the linker chain sequence of glycosaminoglycans (Asp et al. 1969). Defects in GLB1 causes the lysosomal storage diseases GM1 gangliosidosis (Yoshida et al. 1991) and Morquio syndrome B (Oshima et al. 1991).
Reaction - small molecule participants:
Gal [lysosomal lumen]
D-xylose [lysosomal lumen]
H2O [lysosomal lumen]
linker chain(2) [lysosomal lumen]
Reactome.org reaction link: R-HSA-2090079
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Reaction input - small molecules:
water
beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
Reaction output - small molecules:
alpha-D-galactose
aldehydo-D-xylose
Reactome.org link: R-HSA-2090079