Abnormal gangliosides in Tay-Sachs disease, Niemann-Pick's disease, and gargoylism.
The molar ratios of N-acetyl neuraminic acid, hexose, hexosamine, and sphingosine have been determined for the abnormal ganglioside in Tay-Sachs disease that was previously detected as a fast-moving band in thin-layer chromatography, and in two abnormal fast-moving bands of gangliosides from the cortex and white matter of the brain in cases of gargoylism and Niemann-Pick's disease. The fastest-moving ganglioside band in these two conditions contains neither hexosamine nor glucose.
D000605:Amino Alcohols / Q000032:analysis; D001923:Brain Chemistry; D002540:Cerebral Cortex / Q000032:analysis; D002855:Chromatography, Thin Layer; D005732:Gangliosides / Q000032:analysis / Q000378:metabolism*; D006595:Hexosamines / Q000032:analysis; D006601:Hexoses / Q000032:analysis; D006801:Humans; D008064:Lipidoses / Q000378:metabolism*; D009083:Mucopolysaccharidoses / Q000378:metabolism*; D009438:Neuraminic Acids / Q000032:analysis; D009542:Niemann-Pick Diseases / Q000378:metabolism*; D013109:Sphingomyelins / Q000032:analysis
D000605:Amino Alcohols; D005732:Gangliosides; D006595:Hexosamines; D006601:Hexoses; D009438:Neuraminic Acids; D013109:Sphingomyelins