title: Gangliosidosis with total hexosaminidase deficiency: clinical, biochemical and ultrastructural studies and comparison with conventional cases of Tay-Sachs disease.
mesh_terms: D001706:Biopsy; D001923:Brain Chemistry; D002648:Child; D002855:Chromatography, Thin Layer; D004727:Endothelium / Q000201:enzymology; D005625:Frontal Lobe / Q000201:enzymology; D005732:Gangliosides / Q000378:metabolism*; D006596:Hexosaminidases / Q000378:metabolism*; D006651:Histocytochemistry; D006801:Humans; D002479:Inclusion Bodies / Q000032:analysis; D007728:Kupffer Cells / Q000201:enzymology; D008064:Lipidoses / Q000201:enzymology* / Q000473:pathology; D008055:Lipids / Q000032:analysis; D008099:Liver / Q000201:enzymology; D008247:Lysosomes / Q000201:enzymology; D008854:Microscopy, Electron; D009457:Neuroglia / Q000201:enzymology; D009474:Neurons / Q000201:enzymology
chemical_list: D005732:Gangliosides; D008055:Lipids; D006596:Hexosaminidases