Fatty acid elongation activity in fibroblasts from patients with adrenoleukodystrophy (ALD).
The activities of microsomal fatty acid elongation and cytoplasmic de novo fatty acid synthesis were measured in human cultured skin fibroblasts. Both activities in fibroblasts from normal controls and patients with adrenoleukodystrophy (ALD) were compared and slight but a significant increase of elongation activities in ALD fibroblasts was demonstrated. On the other hand, there were no significant differences in the fatty acid synthetase activities. In this study, we measured microsomal fatty acid elongation activities in the presence of N-ethylmaleimide, which completely inhibited the activity of contaminating fatty acid synthetase and also the degradation of fatty acids, and made accurate determination of the elongation activities possible.
D000326:Adrenoleukodystrophy / Q000378:metabolism*; D000328:Adult; D002478:Cells, Cultured; D002648:Child; D003600:Cytosol / Q000378:metabolism; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000378:metabolism*; D005033:Ethylmaleimide / Q000494:pharmacology; D064429:Fatty Acid Synthases / Q000378:metabolism*; D005227:Fatty Acids / Q000096:biosynthesis*; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D007700:Kinetics; D008861:Microsomes / Q000378:metabolism*; D012867:Skin / Q000378:metabolism*; D013329:Structure-Activity Relationship
D005227:Fatty Acids; D064429:Fatty Acid Synthases; D005033:Ethylmaleimide