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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Beta-galactosidases hydrolyse mobilized GM1 to mobilized GM2

H2O + mobilized GM1 → Gal + mobilized GM2

Reactome
R-HSA-1605624
Compartment
lysosomal lumen · lysosomal membrane
Rhea
None recorded

Inputs

Outputs

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. Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP J Biol Chem, 2000
  4. GM1 gangliosidosis: review of clinical, molecular, and therapeutic aspects Mol Genet Metab, 2008