A disorder of ganglioside metabolism with storage of ceramide lactoside, monosialo ceramide lactoside and Tay-Sachs ganglioside in the brain
Pilz, H.; Sandhoff, K.; Jatzkewitz, H.
Journal of Neurochemistry 13(12): 1273-1282
The lipids were studied by thin-layer chromatography and determined quantitatively in the total lipid extract from the formalin-fixed cerebral cortex of a 29-month-old child. In comparison with the normal, increased values were found for ceramide lactoside (Cytolipin H; Cytoside), monosialoceramide lactoside (Haematoside) and Tay-Sachs ganglioside in the proportions 10:2:3. A small amount of glucocerebroside was also found in the pathological cortex. The stored lipids were obtained pure by column chromatography and their chemical constitution determined. The relation of the ceramide lactoside to the normal gangliosides was established by partial and total hydrolysis of samples and determination of the fatty acid composition. The question about the main storage substance and its role in the normal metabolism of the gangliosides, is discussed in light of the chemical changes that occur in gangliosides due to formalin-fixation.