Abstract
After Büchner’s discovery in 18971 that the alcoholic fermentation of glucose could be carried out by yeast-juice (cell free extract of yeast), Harden and Young2 reported in 1904 that the fermentation of glucose by yeast-juice was greatly increased by the addition of boiled filtered yeast-juice, although the boiled yeast-juice itself was incapable of setting up fermentation. This was the first demonstration of the requirement of an “activator” (co-enzyme) for an enzymic reaction. The biological importance of co-enzymes has been well appreciated, since most water soluble vitamins are components of co-enzymes. Until recently, it had not occurred to biochemists that hydrolysis of glycosphingolipids may require a co-factor. Through the work of many laboratories, it has been well recognized that the hydrolysis of sugar chains in glycosphingolipids cannot proceed by the action of enzymes alone; the hydrolysis requires the presence of an activator protein. In this chapter we would like to give a brief overview on activator proteins related to the catabolism of glycosphingolipids and to use our studies on chemical pathology of Type-AB GM2-gangliosidosis as an example to illustrate the biological importance of the activator proteins
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© 1984 Plenum Press, New York
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Li, YT., Li, SC. (1984). Activator Proteins for the Catabolism of Glycosphingolipids. In: Ledeen, R.W., Yu, R.K., Rapport, M.M., Suzuki, K. (eds) Ganglioside Structure, Function, and Biomedical Potential. Advances in Experimental Medicine and Biology, vol 174. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1200-0_18
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DOI: https://doi.org/10.1007/978-1-4684-1200-0_18
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