Studies on lipids from liver and spleen of a child (O.L.) with Niemann-Pick's disease type C.
Large accumulations of sphingomyelin and bis(monoacylglycero)phosphate were found in the spleen of a child, who died from a lipidosis (Morbus Niemann-Pick). The enrichment of both phospholipids in this organ was more extensive than in liver. Moreover: bis(monoacylglycero)phosphate, the lipid characteristic for lysosomes, had a remarkably high content of oleic acid: 76% in spleen and 63% in liver.
D002648:Child; D005227:Fatty Acids / Q000032:analysis; D005260:Female; D006801:Humans; D008055:Lipids / Q000032:analysis*; D008099:Liver / Q000032:analysis*; D008246:Lysophospholipids; D050178:Monoglycerides; D009542:Niemann-Pick Diseases / Q000378:metabolism*; D019301:Oleic Acid; D009829:Oleic Acids / Q000032:analysis; D010712:Phosphatidic Acids / Q000032:analysis; D010743:Phospholipids / Q000032:analysis; D013154:Spleen / Q000032:analysis*
D005227:Fatty Acids; D008055:Lipids; D008246:Lysophospholipids; D050178:Monoglycerides; D009829:Oleic Acids; D010712:Phosphatidic Acids; D010743:Phospholipids; C012786:bis(monoacylglyceryl)phosphate; D019301:Oleic Acid