Document Detail

Fatty acid transport in multiple carboxylase deficiency fibroblasts.
MedLine Citation:
PMID:  2123277     Owner:  NLM     Status:  MEDLINE    
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.
S Packman; S Whitney
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Journal of inherited metabolic disease     Volume:  13     ISSN:  0141-8955     ISO Abbreviation:  J. Inherit. Metab. Dis.     Publication Date:  1990  
Date Detail:
Created Date:  1991-01-10     Completed Date:  1991-01-10     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7910918     Medline TA:  J Inherit Metab Dis     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  716-20     Citation Subset:  IM    
Department of Pediatrics, University of California, San Francisco 94143.
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MeSH Terms
Arachidonic Acid
Arachidonic Acids / metabolism
Biological Transport, Active
Biotin / metabolism
Carboxy-Lyases / deficiency*
Cell Line
Culture Media
Fatty Acids / metabolism*
Fibroblasts / enzymology,  metabolism*
Oleic Acid
Oleic Acids / metabolism
Palmitic Acid
Palmitic Acids / metabolism
Grant Support
Reg. No./Substance:
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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