As GM1 is predominantly broken down in lysosomes by β-galactosidase, mutations in glb1 causes massive accumulation of GM1 and GM1-gangliosidosis, associated with neurodegeneration, so the precise control of the synthesis and degradation of GM1, and related gangliosides, appears critical for their multiple functions. This evidence concerns the gene GLB1 and GM1 gangliosidosis.