Galactolipid fatty acid composition in adrenoleukodystrophy.
The non-hydroxy and hydroxy fatty acids of the major brain galactosphingolipids, cerebroside and sulphatide, have been isolated from white matter, gray matter and myelin of 2 children with adrenoleukodystrophy and from a corresponding control. In addition, cerebroside fatty acids were recovered from a myelin-related fraction on 1 patient. In comparison to control observations, myelin and the myelin-related fraction cerebroside demonstrate a loss of C24:1 and C24h:o fatty acids from normal and hydroxy fatty acid fractions, respectively. White and gray matter galactolipids isolated from 1 patient indicated a signficant increase in short chain (C16, C18 and C18:1) non-hydroxy fatty acids. In the isolated diseased myelin, the ratio of cerebroside hydroxy to non-hydroxy fatty acids was elevated about 1.5 times above the value for normal myelin, whereas the ratio derived for the myelin-related fraction was about two-thirds the values for the control myelin cerebroside.
D000309:Adrenal Insufficiency / Q000378:metabolism; D001923:Brain Chemistry; D001927:Brain Diseases / Q000378:metabolism*; D002554:Cerebrosides / Q000032:analysis*; D002648:Child; D005227:Fatty Acids / Q000032:analysis*; D006801:Humans; D009186:Myelin Sheath / Q000032:analysis; D013433:Sulfoglycosphingolipids / Q000032:analysis*
D002554:Cerebrosides; D005227:Fatty Acids; D013433:Sulfoglycosphingolipids