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Structures of gangliosides GM3, GM2, GD3, and GD1a. Only one of the various lipoforms is shown. GD1a is the most abundant ganglioside in the adult human brain. Ganglioside GM2 is the major storage substance in Tay-Sachs disease (β-hexosaminidase α-subunit deficiency), Sandhoff disease (β-hexosaminidase β-subunit deficiency), and GM2-activator deficiency.
