Biochemical study on myelin in adrenoleukodystrophy.
Two myelin fractions, heavy and light, were isolated from white matter of a patient with adrenoleukodystrophy. Morphologically, heavy myelin fraction showed compact lamellar structures, and light myelin fraction consisted of loose lamellar structures and rod-like clear space. Analytical studies demonstrated that the chemical composition of heavy myelin fraction was almost the same as that of normal myelin and that light myelin fraction consisted mainly of cholesterol. Long chain saturated fatty acids, C25:0 and C26:0, were increased and C24:1 was decreased in sphingoglycolipids of heavy myelin fraction of the patient.
D001923:Brain Chemistry; D002648:Child; D003711:Demyelinating Diseases / Q000473:pathology*; D005227:Fatty Acids / Q000032:analysis; D006017:Glycolipids / Q000032:analysis; D006028:Glycosphingolipids / Q000032:analysis; D006801:Humans; D006880:Hydroxy Acids / Q000032:analysis; D008297:Male; D008563:Membrane Lipids / Q000032:analysis; D009186:Myelin Sheath / Q000032:analysis* / Q000648:ultrastructure; D010743:Phospholipids / Q000032:analysis; D013261:Sterols / Q000032:analysis
D005227:Fatty Acids; D006017:Glycolipids; D006028:Glycosphingolipids; D006880:Hydroxy Acids; D008563:Membrane Lipids; D010743:Phospholipids; D013261:Sterols