Very long-chain fatty acids in neutral lipids and glycerophospholipids of adrenoleukodystrophy-cultured skin fibroblasts.
Three major lipid fractions, i.e., cholesterol ester, triacylglycerol and glycerophospholipids, were investigated cultured fibroblasts to clarify whether very long-chain fatty acids were accumulated or not. Abnormally accumulated very long-chain fatty acids were confirmed in all three lipid fractions. Our results support our previous report that there may be a generalized abnormal metabolism of very long-chain fatty acids in adrenoleukodystrophy.
D000326:Adrenoleukodystrophy / Q000235:genetics / Q000378:metabolism*; D002353:Carrier State / Q000378:metabolism; D002478:Cells, Cultured; D002788:Cholesterol Esters / Q000378:metabolism; D002851:Chromatography, High Pressure Liquid; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism*; D006801:Humans; D010743:Phospholipids / Q000378:metabolism*; D012867:Skin / Q000166:cytology; D014280:Triglycerides / Q000378:metabolism
D002788:Cholesterol Esters; D005227:Fatty Acids; D010743:Phospholipids; D014280:Triglycerides; C017364:hexacosanoic acid