Alterations in brain gangliosides and other lipids of patients with Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis (SSPE).
Lipid composition was examined with cerebral cortices of three cases of Creutzfeldt-Jakob (C-J) disease and one case of subacute sclerosing panencephalitis (SSPE). Phosphatidylethanolamine decreased in both C-J and SSPE brains. Sphingomyelin increased in CJ. The levels of cholesterol esters which occurred in a trace amount in normal control were elevated in C-J and more severely in SSPE. The total amounts of gangliosides were reduced in C-J and SSPE. Gangliosides GD1b, GT1b and GQ1b in the biosynthetic pathway B [23] specifically decreased in C-J, while GM1 and GD3 increased. An increased content of GD3 may be related to the proliferation of reactive astrocytes. A decrease of 22:6 (n-3) accompanied with an increase of oleic acid in the fatty acid composition of the acidic phospholipid fraction was observed. This might be a cause for alterations in membrane function.
D000328:Adult; D000368:Aged; D002540:Cerebral Cortex / Q000032:analysis*; D002648:Child; D002784:Cholesterol / Q000032:analysis; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism*; D005227:Fatty Acids / Q000032:analysis; D005260:Female; D005732:Gangliosides / Q000172:deficiency*; D006801:Humans; D008055:Lipids / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D010714:Phosphatidylethanolamines / Q000032:analysis; D013109:Sphingomyelins / Q000032:analysis; D013344:Subacute Sclerosing Panencephalitis / Q000378:metabolism*
D005227:Fatty Acids; D005732:Gangliosides; D008055:Lipids; D010714:Phosphatidylethanolamines; D013109:Sphingomyelins; D002784:Cholesterol