title: Adrenoleukodystrophy. Accumulation of cholesterol esters with very long chain fatty acids.
abstract: An accumulation of fatty acids having chain lengths of C22 to C26 was demonstrated in the cholesterol ester fraction of white matter from two boys with adrenoleukodystrophy. A similar accumulation of very long chain fatty acids was also shown in gangliosides and cerebrosides. A disorder of the one-carbon degradation system responsible for the metabolism of some of the very long chain fatty acids may be the basic enzymatic defect in this condition.
mesh_terms: D000307:Adrenal Gland Diseases / Q000378:metabolism*; D001921:Brain / Q000378:metabolism*; D001923:Brain Chemistry; D001927:Brain Diseases / Q000378:metabolism*; D002648:Child; D002788:Cholesterol Esters / Q000032:analysis / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D005732:Gangliosides / Q000032:analysis; D006801:Humans; D008297:Male
chemical_list: D002788:Cholesterol Esters; D005227:Fatty Acids; D005732:Gangliosides