Fatty acid patterns in brain, fibroblast, leukocyte and body fluid lipids in adrenoleukodystrophy.
Fatty acid patterns of certain tissue and body fluid lipids of ALD and control cases were investigated by thin layer and gas chromatography. A high percentage (55.2%) of VLFA was found in the ALD brain white matter cholesterol esters; this was mainly due to increased proportions of 26:0, 25:0, 26:1 and greater than 26 C fatty acids. The percentage of VLFA in the white matter cholesterol esters of pathological controls with myelin breakdown of different etiology was low (0.4-7.1%). In the fibroblast total lipids of an ALD patient an increase of 26:0 and 25:0 fatty acids was found. Moderately increased proportions of hexacosanoic acid were observed in the leukocyte total lipids of 2 severely affected ALD patients, but not in a slightly as well as in a possible affected case. No accumulation of VLFA was detected in cholesterol ester, triglyceride and free fatty acid fractions in the CSF of an ALD patient and in ALD serum sphingomyelin. Our results substantiate the specificity of VLFA accumulation in the white matter cholesterol esters of ALD brain and lend some further evidence to the view that a generalized impairment of VLFA metabolism seems to be present in this disease.
D000293:Adolescent; D000309:Adrenal Insufficiency / Q000378:metabolism*; D001826:Body Fluids / Q000378:metabolism; D001921:Brain / Q000378:metabolism; D001928:Brain Diseases, Metabolic / Q000378:metabolism*; D002648:Child; D002788:Cholesterol Esters / Q000378:metabolism; D003711:Demyelinating Diseases / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D005230:Fatty Acids, Nonesterified / Q000378:metabolism; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D007962:Leukocytes / Q000378:metabolism; D009185:Myelin Proteins / Q000378:metabolism
D002788:Cholesterol Esters; D005227:Fatty Acids; D005230:Fatty Acids, Nonesterified; D009185:Myelin Proteins