Accumulation of glyceryl ether lipids in Wolman's disease

• Main Authors: H J Lin, M S Lie Ken Jie, F C Ho
• Format: Article
• Language: English
• Published: Elsevier 1976-01-01
• Series: Journal of Lipid Research
• Subjects: lipid storage disease; alkyl-diacylglycerol; adrenal lipids
• Online Access: http://www.sciencedirect.com/science/article/pii/S0022227520370164

We have shown that ether-linked glycerolipids accumulated in the adrenal, liver, and spleen of a male Chinese infant with Wolman's disease; the increases were mainly in the alkyl and alk-1-enyl glycerolipids that did not contain phosphorus. Alkyldiacylglycerol accounted for a portion of the rise in the neutral alkylglycerols. The spleen also contained increased amounts of ether-linked phosphoglycerides of the alkyl and alk-1-enyl types. Organs from a Niemann-Pick patient were also included in this study; they did not show comparable rises in the content of ether-linked glycerolipids, suggesting the possibility that storage of these compounds may be characteristic of Wolman's disease, or a variant form thereof.