| Lovastatin and sodium phenylacetate normalize the levels of very long chain fatty acids in skin fibroblasts of X- adrenoleukodystrophy. | |
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MedLine Citation:
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PMID: 9600263 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The present study underlines the importance of lovastatin, an inhibitor of 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase, and the sodium salt of phenylacetic acid (NaPA), an inhibitor of mevalonate pyrophosphate decarboxylase, in normalizing the pathognomonic accumulation of saturated very long chain fatty acids (VLCFA) in cultured skin fibroblasts of X-adrenoleukodystrophy (X-ALD) in which the ALD gene is either mutated or deleted. Lovastatin or NaPA alone or in combination stimulated the beta-oxidation of lignoceric acid (C24:0) and normalized the elevated levels of VLCFA in skin fibroblasts of X-ALD. Ability of lovastatin and NaPA to normalize the pathognomonic accumulation of VLCFA in skin fibroblasts of X-ALD may identify these drugs as possible therapeutics for X-ALD. |
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Authors:
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I Singh; K Pahan; M Khan |
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Publication Detail:
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Type: Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: FEBS letters Volume: 426 ISSN: 0014-5793 ISO Abbreviation: FEBS Lett. Publication Date: 1998 Apr |
Date Detail:
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Created Date: 1998-06-10 Completed Date: 1998-06-10 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0155157 Medline TA: FEBS Lett Country: NETHERLANDS |
Other Details:
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Languages: eng Pagination: 342-6 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Medical University of South Carolina, Charleston 29425, USA. singhi@musc.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adrenoleukodystrophy
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drug therapy,
genetics,
metabolism* Cells, Cultured Drug Synergism Fatty Acids / metabolism* Fibroblasts / drug effects, metabolism, pathology Gene Deletion Humans Intracellular Fluid / chemistry, drug effects Lovastatin / pharmacology* Mevalonic Acid / antagonists & inhibitors Oxidation-Reduction / drug effects Phenylacetates / pharmacology* Skin / metabolism*, pathology X Chromosome / genetics* |
| Grant Support | |
ID/Acronym/Agency:
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NS-22576/NS/NINDS NIH HHS; NS-34741/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Fatty Acids; 0/Phenylacetates; 103-82-2/phenylacetic acid; 150-97-0/Mevalonic Acid; 557-59-5/lignoceric acid; 75330-75-5/Lovastatin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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