Reaction: GLB1 hydrolyses GM2A:GA1 to GM2A:GA2
- in pathway: Glycosphingolipid catabolism
Ganglioside GA1 (asialo-GM1, ganglio-N-tetraosylceramide) is a ganglioside that accumulates in nerve cells in GM1-gangliosidosis, showing that GA1 is hydrolyzed by GLB1, probably to GA2 (Caciotti et al., 2005). Since GLB1 acts on solubilized gangliosides, we assume GA1 and GA2 are bound to GM2A during the process.
Reaction - small molecule participants:
Gal [lysosomal lumen]
H2O [lysosomal lumen]
Reactome.org reaction link: R-HSA-9840795
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Reaction input - small molecules:
water
Reaction output - small molecules:
alpha-D-galactose
Reactome.org link: R-HSA-9840795