Document Detail

Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts.
MedLine Citation:
PMID:  9469587     Owner:  NLM     Status:  MEDLINE    
The relationship between peroxisomal and mitochondrial oxidation of the methyl branched fatty acids, phytanic acid and pristanic acid, was studied in normal and mutant human skin fibroblasts with established enzyme deficiencies. Tandem mass spectrometry was used for analysis of the acylcarnitine intermediates. In normal cells, 4,8-dimethylnonanoylcarnitine (C11:0) and 2,6-dimethylheptanoylcarnitine (C9:0) accumulated after incubation with either phytanic acid or pristanic acid. These intermediates were not observed when peroxisome-deficient cells from Zellweger patients were incubated with the same compounds, pointing to the involvement of peroxisomes in the formation of these acylcarnitine intermediates. Similar experiments with fibroblasts deficient in carnitine palmitoyltransferase I, carnitine-acylcarnitine translocase or carnitine palmitoyltransferase II revealed that mitochondrial carnitine palmitoyltransferase I is not required for the oxidation of phytanic acid or pristanic acid, whereas both carnitine-acylcarnitine translocase and carnitine palmitoyltransferase II are necessary. These studies demonstrate that both phytanic acid and pristanic acid are initially oxidized in peroxisomes to 4,8-dimethylnonanoyl-CoA, which is converted to the corresponding acylcarnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. After transport across the mitochondrial membrane and transfer of the acylgroup to coenzyme A, further oxidation to 2,6-dimethylheptanoyl-CoA occurs.
N M Verhoeven; D S Roe; R M Kok; R J Wanders; C Jakobs; C R Roe
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of lipid research     Volume:  39     ISSN:  0022-2275     ISO Abbreviation:  J. Lipid Res.     Publication Date:  1998 Jan 
Date Detail:
Created Date:  1998-03-26     Completed Date:  1998-03-26     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0376606     Medline TA:  J Lipid Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  66-74     Citation Subset:  IM    
Department of Clinical Chemistry, Free University Hospital, Amsterdam, The Netherlands.
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MeSH Terms
Carnitine Acyltransferases / deficiency,  metabolism
Carnitine O-Palmitoyltransferase / deficiency,  metabolism
Cells, Cultured
Fatty Acids / metabolism*
Fibroblasts / enzymology*
Gas Chromatography-Mass Spectrometry
Mass Spectrometry
Microbodies / enzymology*
Mitochondria / enzymology*
Phytanic Acid / metabolism*
Zellweger Syndrome / enzymology
Reg. No./Substance:
0/Fatty Acids; 1189-37-3/pristanic acid; 14721-66-5/Phytanic Acid; EC 2.3.1.-/Carnitine Acyltransferases; EC O-Palmitoyltransferase

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