Accumulation of glyceryl ether lipids in Wolman's disease.
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.
D000311:Adrenal Glands / Q000378:metabolism; D000328:Adult; D002648:Child; D004987:Ethers / Q000378:metabolism; D005260:Female; D005990:Glycerol / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D050356:Lipid Metabolism; D008064:Lipidoses / Q000235:genetics / Q000378:metabolism; D008055:Lipids / Q000302:isolation & purification; D008099:Liver / Q000378:metabolism; D009542:Niemann-Pick Diseases / Q000378:metabolism; D013154:Spleen / Q000378:metabolism; D013577:Syndrome; D014973:Xanthomatosis / Q000235:genetics* / Q000378:metabolism
D004987:Ethers; D008055:Lipids; D005990:Glycerol