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The lipid composition of autopsy specimens from a patient suffering from Farber's disease (clinically verified) has been characterized and correlated to those of normal adult, infant, and foetal specimens. In autopsy specimens from the brain and liver tissue of the patient in question, an increased amount of an unknown glycolipid fraction and a phosphoglyceride fraction was found when the data was correlated to those of foetal and infant tissues. The glycolipid fraction was isolated by column chromatography and characterized chemically. This lipid was shown to contain glucose and galactosamine in the proportion of 4:1. Gas chromatography of the glycolipid revealed the presence of only shorter chain fatty acids, but only minor quantities of the C:22-C:24 series. In addition, quantitative estimation of lipid soluble sialic (ganglioside) revealed about 30 per cent increase in sialic acid (ganglioside) in total brain tissue, but about 30 per cent decrease in liver tissue. The data obtained are discussed on the basis of the available literature.
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Clausen et al. (1970) studied this question.
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