Ultrastructural lipid and glycoconjugate cytochemistry of membranous lipodystrophy (Nasu-Hakola disease)

Abstract

Summary In order to assess the lipid and glycoconjugate characteristics of membranous lipodystrophy, a 29-year-old male with this disease was studied using an ultrastructural cytochemical approach. The specific membranocystic lesions of the disease are composed of cystic spaces and the lining membranes. The membranes were observed to have a two-layered character: microtubular structures in the layer adjacent to the spaces and a central amorphous zone. Lipid staining and the lipase digestion test revealed triglycerides localized not only in the cystic spaces but also in the microtubular structures. Lectin histochemical examintion of carbohydrate components demonstrated thatMaclura pomifera agglutinin bound strongly to the membranes, whileGriffonia simplicifolia I,G. simplicifolia II,Concanavalia ensiformis andTriticum vulgaris agglutinin reacted weakly. Our results indicate the presence of triglycerides and carbohydrates with mainlyα-D-galactose residues in the distinctive membranocystic lesions, in particular in the microtubular structures.