Lysosomal non-lipid component of Gaucher's cells

Abstract

An ultrastructural, histochemical and chemical analysis of storage elements in the infantile form of Gaucher's disease showed that in addition to cerebroside the lysosomes also included a non-lipid component of protein, or possibly glycoprotein nature. This component, easily removable with trypsin, was present in such quantities that it conditioned the typical solid and fibrillar appearance of storage elements even after they had been substantially delipidized. Another noteworthy finding was that the ultrastructural appearance of tubular structures generally regarded as stored cerebrosides persisted in all the extracted specimens without any noticeable change. The findings are compared with available data from the literature and their significance briefly discussed.