| Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids. | |
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MedLine Citation:
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PMID: 3382393 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A method was developed to prepare peroxisome-enriched fractions depleted of microsomes and mitochondria from cultured skin fibroblasts. The method consists of differential centrifugation of a postnuclear supernatant followed by density gradient centrifugation on a discontinuous Metrizamide gradient. The activity of hexacosanoyl-CoA synthetase was subsequently measured in postnuclear supernatants and peroxisome-enriched fractions prepared from cultured skin fibroblasts from control subjects and patients with X-linked adrenoleukodystrophy. Whereas the hexacosanoyl-CoA synthetase activity in postnuclear supernatants of X-linked adrenoleukodystrophy fibroblasts was only slightly decreased (77.8 +/- 4.4% of control (n = 15], enzyme activity was found to be much more markedly reduced in peroxisomal fractions isolated from the mutant fibroblasts (19.6 +/- 6.7% of control (n = 5]. This is a direct demonstration that the defect in X-linked adrenoleukodystrophy is at the level of a deficient ability of peroxisomes to activate very long chain fatty acids, as first suggested by Hashmi et al. [Hashmi, M., Stanley, W. and Singh, I. (1986) FEBS Lett. 86, 247-250]. |
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Authors:
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R J Wanders; C W van Roermund; M J van Wijland; R B Schutgens; H van den Bosch; A W Schram; J M Tager |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Biochemical and biophysical research communications Volume: 153 ISSN: 0006-291X ISO Abbreviation: Biochem. Biophys. Res. Commun. Publication Date: 1988 Jun |
Date Detail:
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Created Date: 1988-07-22 Completed Date: 1988-07-22 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0372516 Medline TA: Biochem Biophys Res Commun Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 618-24 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, University Hospital Amsterdam, The Netherlands. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenoleukodystrophy
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metabolism* Catalase / metabolism Cell Fractionation Cells, Cultured Centrifugation, Density Gradient Diffuse Cerebral Sclerosis of Schilder / metabolism* Fatty Acid Synthetase Complex / metabolism Fatty Acids / metabolism* Humans Microbodies / metabolism* Microsomes / metabolism Mitochondria / metabolism Skin / enzymology |
| Chemical | |
Reg. No./Substance:
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0/Fatty Acids; 506-46-7/hexacosanoic acid; EC 1.11.1.6/Catalase; EC 6.-/Fatty Acid Synthetase Complex |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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