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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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GLB1 hydrolyses a glycosaminoglycan

H2O + Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal → Gal + GlcNAc(S)-Gal-GlcNAc(S)-Gal

Reactome
R-HSA-1630306
Compartment
lysosomal lumen
Rhea
None recorded

Inputs

  • H2OCHEBI:15377, H2O, lysosomal lumen
  • Gal-GlcNAc(S)-Gal-GlcNAc(S)-GalCHEBI:63850, C34H58N2O31S2, lysosomal lumen

Outputs

  • GalCHEBI:28061, C6H12O6, lysosomal lumen
  • GlcNAc(S)-Gal-GlcNAc(S)-GalCHEBI:63851, C28H48N2O27S2, lysosomal lumen

Catalysed by

  • beta-galactosidase activity of BGAL [lysosomal lumen]EC 3.2.1.23

Literature

  1. Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase Biochem J, 1969
  2. Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases Am J Hum Genet, 1991
  3. Human beta-galactosidase gene mutations in morquio B disease Am J Hum Genet, 1991