Characterization by lectin binding of the sugar moiety of glycocompounds stored in inherited diseases.
Sleutelwoorden
Abstract
Kidney and liver samples from two cases of Fabry's disease and spleen and liver samples from Gaucher and Niemann-Pick diseases were tested for binding to lectins such as peanut agglutinin (PNA), Bandeiraea simplicifolia, (BSA), canavalia ensiformis (Con A), soybean agglutinin (SBA) and wheat germ agglutinin (WGA) labelled with horseradish peroxidase using histochemical techniques. These techniques allowed the localization of compounds with alpha-galactosyl residues in tissues from Fabry's disease. In tissues from the Gaucher and Niemann-Pick cases, the storage material was found to be more complex than expected, and some problems regarding the significance of lectin binding are discussed.