Chemical studies in gargoylism.

Abstract

) have contributed to our knowledge of the metabolism of different glycolipids. Thus, a low concentration of 3-glucosidase in human brain and other organs will give rise to a blocked degrada- tion of gangliosides, with accumulation of gluco- cerebrosides, the disease associated with this enzymatic defect having previously been named Gaucher's disease (Gaucher, 1882; Philippart and Menkes, 1964; Brady, Kanfer, and Shapiro, 1965a, b; Svennerholm, 1966).