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
- H2OCHEBI:15377, H2O, lysosomal lumen
- mobilized GM1R-HSA-9840644, lysosomal lumen
Outputs
- GalCHEBI:28061, C6H12O6, lysosomal lumen
- mobilized GM2R-HSA-9840646, lysosomal lumen
Catalysed by
- beta-galactosidase activity of BGAL [lysosomal lumen]EC 3.2.1.23
Literature
- Human small-intestinal beta-galactosidases. Separation and characterization of one lactase and one hetero beta-galactosidase Biochem J, 1969
- Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases Am J Hum Genet, 1991
- Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP J Biol Chem, 2000
- GM1 gangliosidosis: review of clinical, molecular, and therapeutic aspects Mol Genet Metab, 2008