title: Histochemical study of Hurler's disease by the use of peroxidase-labelled lectins.
abstract: Peroxidase-labelled lectins specific for various carbohydrate residues were used as histochemical reagents in the investigation of Hurler's syndrome. Peanut lectin was used to detect terminal D-galactose, wheatgerm lectin for N-acetyl-D-glucosamine, soybean lectin for N-acetyl-D-galactosamine, Tetragonolobus lotus lectin for alpha-L-fucose and Bandeiraea S. lectin for alpha-D-galactose. It was found that Kupffer cells in the liver and splenic reticulo-endothelial cells contain acid mucopolysaccharides which bind lectins in paraffin sections after appropriate fixation. The pattern of lectin binding suggests that such cells contain significant amounts of D-galactose, L-fucose, N-acetyl-D-galactosamine and N-acetyl-D-glucosamine. It is likely that the last named carbohydrate is present as a polymer. Neurones contain a different carbohydrate, rich in galactose and fucose but poor in N-acetyl-D-glucosamine. This compound is resident to lipid extraction. Hepatocytes, as a rule, do not react with lectins, most likely because of loss of the more soluble mucopolysaccharides during fixation. The results are consistent with the biochemical data of Hurler's syndrome and indicate that lectins can be a useful tool for the investigation of the cytochemistry of storage disorders.
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006651:Histocytochemistry / Q000379:methods; D006801:Humans; D037102:Lectins; D008099:Liver / Q000473:pathology; D008059:Mucopolysaccharidosis I / Q000473:pathology*; D019887:Peanut Agglutinin; D010544:Peroxidases; D037121:Plant Lectins; D030262:Soybean Proteins; D013116:Spinal Cord / Q000473:pathology; D014909:Wheat Germ Agglutinins; D010835:Phytohemagglutinins
chemical_list: D037102:Lectins; D019887:Peanut Agglutinin; D010544:Peroxidases; D037121:Plant Lectins; D030262:Soybean Proteins; D014909:Wheat Germ Agglutinins; C028824:soybean lectin; D010835:Phytohemagglutinins