[Glycolipid analysis by confocal laser scanning microscopic system].
The combined use of immunofluorescence method with oligosaccharide-specific monoclonal antibodies against glycolipids and a confocal laser scanning microscopic system was successful to detect the storage of glycolipids in cultured cells derived from patients with lysosomal diseases. The accumulated glycolipids were observed stereoscopically as granular inclusions in the cells. An immunofluorometric semiquantative determination was achieved of globotriaosylceramide in cultured fibroblasts from Fabry disease patients or GM2-ganglioside in cultured amniocytes from Tay-Sachs disease. Heterozygote identification of the former disease or prenatal diagnosis of the latter one were confirmed by counting immunoreactive cells or by digital imaging analysis. The present immunofluorescence method is applicable to the diagnosis of the other lipidosis with accumulation of the specific glycolipids.
Itoh
K
K
Tokyo Metropolitan Institute of Medical Science, Department of Clinical Genetics.
D000818:Animals; D000911:Antibodies, Monoclonal; D002478:Cells, Cultured; D005455:Fluorescent Antibody Technique; D006017:Glycolipids / Q000032:analysis*; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000175:diagnosis / Q000378:metabolism*; D018613:Microscopy, Confocal
D000911:Antibodies, Monoclonal; D006017:Glycolipids