Application Notes:
As this product is derived from a natural source, there may be variations in the sphingoid backbone.
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). A mutation in the GM2A gene results in GM2 activator deficiency that also leads to accumulation of GM2.4
Selected References:
1. L. Svennerholm, et al. (eds.), Structure and Function of Gangliosides, New York, Plenum, 1980
2. T. Kolter, R. Proia, K. Sandhoff “Combinatorial Ganglioside Biosynthesis” J. Biol. Chem., Vol. 277, No. 29, pp. 25859-25862, 2002
3. S. Birkle, G. Zeng, L. Gao, R.K. Yu, and J. Aubry “Role of tumor-associated gangliosides in cancer progression” Biochimie, Vol. 85 pp. 455–463, 2003
4. R. Gravel et al., The Metabolic and Molecular Bases of Inherited Disease (C. R. Scriver, W. S. Sly, B. Childs, A. L. Beaudet, D. Valle, K. W. Kinzler, and B. Vogelstein, eds) pp. 3827–3876, McGraw-Hill Inc., New York, 2001