Histochemical and ultrastructural study of Gaucher cells.

Findings are presented of the histochemical and electron microscopical studies of several bioptic specimens of a juvenile form of Gaucher's disease. The storage was restricted to the macrophages with the exception of occasional hepatocytes in which structures closely resembling the storage tubules were found. The cytoplasmic striations of GC were found to be composed both of lipid and a glycoprotein components which can be easily dissociated from each other by means of either organic solvent extraction or proteolytic digestion. According to the results obtained it is the glycoprotein component which is mainly responsible for the cytoplasmic striations. On the contrary the majority of the tubular storage material of the Krabbe cells was found to be removed with chloroform methanol. Results of the enzyme histochemical study (hydrolases and dehydrogenases) point to the considerably higher overall metabolic activity of GC when compared to the Niemann-Pick storage macrophages.