| Fatty acid transport in multiple carboxylase deficiency fibroblasts. | |
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MedLine Citation:
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PMID: 2123277 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Holocarboxylase synthetase (HS) and biotinidase deficiencies have been identified as causes of biotin-responsive multiple carboxylase deficiency. Acetyl-CoA carboxylase (ACC) deficiency has been shown to occur in multiple carboxylase deficiency, and HS(-) fibroblasts are being employed to investigate compensatory regulatory responses in cells deficient in ACC. In previous studies, biotin starved HS(-) fibroblasts showed a reduced fatty acid content, an abnormal percentage composition of fatty acids, and a preservation of longer-chain fatty acid contents of cells. We herein ask whether the mutant cells show compensatory increases in the transport of longer-chain fatty acids from the medium into fibroblasts. In the present experiments there was no change in the uptake of arachidonate, palmitate or oleate following growth of mutant and control fibroblasts in (+) or (-) biotin conditions. Differential fatty acid uptake from the medium is therefore not a compensatory mechanism in HS(-) cells, and cannot account for the specific changes in fatty acid composition produced by biotin restriction. |
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Authors:
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S Packman; S Whitney |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: 13 ISSN: 0141-8955 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 1990 |
Date Detail:
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Created Date: 1991-01-10 Completed Date: 1991-01-10 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: NETHERLANDS |
Other Details:
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Languages: eng Pagination: 716-20 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, University of California, San Francisco 94143. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Arachidonic Acid Arachidonic Acids / metabolism Biological Transport, Active Biotin / metabolism Carboxy-Lyases / deficiency* Cell Line Culture Media Fatty Acids / metabolism* Fibroblasts / enzymology, metabolism* Humans Mutation Oleic Acid Oleic Acids / metabolism Palmitic Acid Palmitic Acids / metabolism |
| Grant Support | |
ID/Acronym/Agency:
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AM31366/AM/NIADDK NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Arachidonic Acids; 0/Culture Media; 0/Fatty Acids; 0/Oleic Acids; 0/Palmitic Acids; 112-80-1/Oleic Acid; 506-32-1/Arachidonic Acid; 57-10-3/Palmitic Acid; 58-85-5/Biotin; EC 4.1.1.-/Carboxy-Lyases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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