Lectin histochemistry of gangliosidosis

Summary Lectin histochemical studies were performed on selected formalin-fixed, paraffin-embedded tissues of patients affected with the O variant of G_M2-gangliosidosis (i.e., Sandhoff's disease). The purpose was to identify specific sugar residues of undegraded stored substances in cytoplasm of affected cells. We studied neural tissues from 13 patients, visceral tissues from four patients, and placentae from three affected fetuses. Neurons in all 13 cases studied stained withConcanavalia ensiformis agglutinin (Con A) and withUlex europaeus agglutinin-I (UEA-I). Succinylated wheat germ agglutinin (S-WGA) stained affected visceral cells and astrocytes and macrophages in the central nervous system. These results demonstrate that -d-mannosyl and -l-fucosyl residues, which bind Con A and UEA-I, respectively, are present in affected neurons. Furthermore, they revealed the affected nonneuronal cells and astrocytes contain complex carbohydrates with nonreducing terminal -N-acetylglucosamine, which binds S-WGA.Supported by grant NS 2176 from the National Institute of Neurological and Communicative Disorders and Stroke