The fine structure of the cerebroside occurring in Gaucher's disease.

Abstract

Gaucher's disease is a metabolic disorder characterized by the accumulation of excessive quantities of glucocerebroside in the reticuloendothelial system.Brady et al. have demonstrated that individuals with this disease have deficient amounts of glucocerebrosidase, one of the enzymes required for complete deg- radation of the lipid of the red cell membrane.'The characteristic cell of Gaucher's disease has numerous membrane-enclosed sacs containing long slender tubules (Fig. 1).Cerebroside extracted with lipid solvents from the tissues of w ..1.-Low-power view of a GauCher cell showing a membrane-enclosed space lying adjacent to the nucleus.The numerous slender tubules are present within this cytoplasmic sac.Magnification, X 16,000.