lyso-Monosialoganglioside GM2 (NH4+ salt), bovine

Gangliosides1 are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially in neuronal cells in the central nervous system.2 They participate in cellular proliferation, differentiation, adhesion, signal transduction, cell-to-cell interactions, tumorigenesis, and metastasis.3 GM2 regulates the function of ciliary neurotrophic factor receptors. The accumulation of GM2 (due to a deficiency in beta-hexosaminidase) has characterized Tay-Sachs disease (due to a mutation in the gene HEXA) and Sandhoff disease (due to a mutation in the gene HEXB).4 lyso-GM2 was also found in elevated amounts in brains with Sandhoff and Tay-Sachs disease. Although the origin of lyso-GM2 remains unknown, it is tied with the loss of HEXA. In a study that subjected lyso-GM2 to modified beta-hexasaminidase (Hex B) which hydrolyzes GM2 and associated gangliosides, Hex B was found to strongly influence the lyso-GM2 levels to decrease.5