Reactome | Beta-galactosidases hydrolyze GM2A:GA1 to GM2A:GA2

Beta-galactosidases hydrolyze GM2A:GA1 to GM2A:GA2

Stable Identifier

R-HSA-9840795

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

ReviewStatus

5/5

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Beta-galactosidases hydrolyze GM2A:GA1 to GM2A:GA2

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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. GLB1L, GLB1L2, and GLB1L3 are gene products with nearly identical protein sequences to GLB1 and may be able to perform the same function.

Literature References

PubMed ID	Title	Journal	Year
15714521	Role of beta-galactosidase and elastin binding protein in lysosomal and nonlysosomal complexes of patients with GM1-gangliosidosis Caciotti, A, Donati, MA, Boneh, A, d'Azzo, A, Federico, A, Parini, R, Antuzzi, D, Bardelli, T, Nosi, D, Kimonis, V, Zammarchi, E, Morrone, A	Hum Mutat	2005