Gangliosides in subacute sclerosing leukoencephalitis: isolation and fatty acid composition of nine fractions.
Gangliosides from brain of an 8 yr old boy with subacute sclerosing leukoencephalitis have been studied in terms of pattern and structure. Thin-layer chromatography showed that both gray and white matter have a highly abnormal pattern, with elevation of the relative proportion of four gangliosides corresponding to minor species in normal brain. The total level of lipid-bound sialic acid, however, was not increased, which indicated a compensating loss of other gangliosides. Two of the proliferating species were monosialogangliosides (G(5) and G(6)) (Korey nomenclature), and two were disialo types (G(2A) and G(3A)). Studies of their carbohydrate structures are described. Nine ganglioside fractions were isolated by preparative TLC in combination with column chromatography, and the fatty acid compositions were determined. Seven contained stearate as the major component, while two (G(3A) and G(6)) had relatively large proportions of oleate and palmitate. Five of the fractions contained two fatty acids of long chain-length and unknown structure.
D001923:Brain Chemistry; D002648:Child; D002845:Chromatography; D002849:Chromatography, Gas; D002854:Chromatography, Paper; D002855:Chromatography, Thin Layer; D002549:Diffuse Cerebral Sclerosis of Schilder; D005227:Fatty Acids / Q000032:analysis*; D005260:Female; D005732:Gangliosides / Q000032:analysis*; D006595:Hexosamines / Q000032:analysis; D006601:Hexoses / Q000032:analysis; D006801:Humans; D008297:Male; D009829:Oleic Acids / Q000032:analysis; D010169:Palmitic Acids / Q000032:analysis; D013229:Stearic Acids / Q000032:analysis
D005227:Fatty Acids; D005732:Gangliosides; D006595:Hexosamines; D006601:Hexoses; D009829:Oleic Acids; D010169:Palmitic Acids; D013229:Stearic Acids