Activator Proteins for the Catabolism of Glycosphingolipids

  • Y.-T. Li
  • S.-C. Li
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 174)


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


Enzymic Hydrolysis Activator Protein Lipid Storage Disease Bovine Spleen Cerebroside Sulfate 
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Copyright information

© Plenum Press, New York 1984

Authors and Affiliations

  • Y.-T. Li
    • 1
  • S.-C. Li
    • 1
  1. 1.Department of BiochemistryDelta Regional Primate Research CenterNew OrleansUSA

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