Document Detail

Peroxisomal fatty acid uptake mechanism in Saccharomyces cerevisiae.
MedLine Citation:
PMID:  22493507     Owner:  NLM     Status:  MEDLINE    
Peroxisomes play a major role in human cellular lipid metabolism, including fatty acid β-oxidation. The most frequent peroxisomal disorder is X-linked adrenoleukodystrophy, which is caused by mutations in ABCD1. The biochemical hallmark of X-linked adrenoleukodystrophy is the accumulation of very long chain fatty acids (VLCFAs) due to impaired peroxisomal β-oxidation. Although this suggests a role of ABCD1 in VLCFA import into peroxisomes, no direct experimental evidence is available to substantiate this. To unravel the mechanism of peroxisomal VLCFA transport, we use Saccharomyces cerevisiae as a model organism. Here we provide evidence that in this organism very long chain acyl-CoA esters are hydrolyzed by the Pxa1p-Pxa2p complex prior to the actual transport of their fatty acid moiety into the peroxisomes with the CoA presumably being released into the cytoplasm. The Pxa1p-Pxa2p complex functionally interacts with the acyl-CoA synthetases Faa2p and/or Fat1p on the inner surface of the peroxisomal membrane for subsequent re-esterification of the VLCFAs. Importantly, the Pxa1p-Pxa2p complex shares this molecular mechanism with HsABCD1 and HsABCD2.
Carlo W T van Roermund; Lodewijk Ijlst; Wiktor Majczak; Hans R Waterham; Hendrik Folkerts; Ronald J A Wanders; Klaas J Hellingwerf
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-04-09
Journal Detail:
Title:  The Journal of biological chemistry     Volume:  287     ISSN:  1083-351X     ISO Abbreviation:  J. Biol. Chem.     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-06-11     Completed Date:  2012-09-21     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  2985121R     Medline TA:  J Biol Chem     Country:  United States    
Other Details:
Languages:  eng     Pagination:  20144-53     Citation Subset:  IM    
Departments of Pediatrics and Clinical Chemistry, Laboratory Genetic Metabolic Diseases, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
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MeSH Terms
ATP-Binding Cassette Transporters / genetics,  metabolism*
Adrenoleukodystrophy / genetics,  metabolism
Biological Transport, Active / physiology
Coenzyme A Ligases / genetics,  metabolism
Fatty Acid Transport Proteins / genetics,  metabolism*
Fatty Acids / genetics,  metabolism*
Multiprotein Complexes / genetics,  metabolism
Peroxisomes / genetics,  metabolism*
Saccharomyces cerevisiae / genetics,  metabolism*
Saccharomyces cerevisiae Proteins / genetics,  metabolism*
Reg. No./Substance:
0/ABCD1 protein, human; 0/ABCD2 protein, human; 0/FAT1 protein, S cerevisiae; 0/Fatty Acid Transport Proteins; 0/Fatty Acids; 0/Multiprotein Complexes; 0/PXA1 protein, S cerevisiae; 0/PXA2 protein, S cerevisiae; 0/Saccharomyces cerevisiae Proteins; EC 6.2.1.-/Coenzyme A Ligases; EC protein, S cerevisiae

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