Document Detail


Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance.
MedLine Citation:
PMID:  21488864     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Peroxisomes are indispensable organelles in higher eukaryotes. They are essential for a number of important metabolic pathways, including fatty acid α- and β-oxidation, and biosynthesis of etherphospholipids and bile acids. However, the peroxisomal membrane forms an impermeable barrier to these metabolites. Therefore, peroxisomes need specific transporter proteins to transfer these metabolites across their membranes. The mammalian peroxisomal membrane harbours three ATP-binding cassette (ABC) transporters. In recent years, significant progress has been made in unravelling the functions of these ABC transporters. There is ample evidence that they are involved in the transport of very long-chain fatty acids, pristanic acid, di- and trihydroxycholestanoic acid, dicarboxylic acids and tetracosahexaenoic acid (C24:6ω3). Surprisingly, only one disease is associated with a deficiency of a peroxisomal ABC transporter. Mutations in the ABCD1 gene encoding the peroxisomal ABC transporter adrenoleukodystrophy protein are the cause for X-linked adrenoleukodystrophy, an inherited metabolic storage disorder. This review describes the current state of knowledge on the mammalian peroxisomal ABC transporters with a particular focus on their function in metabolite transport.
Authors:
Stephan Kemp; Frederica L Theodoulou; Ronald J A Wanders
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  British journal of pharmacology     Volume:  164     ISSN:  1476-5381     ISO Abbreviation:  Br. J. Pharmacol.     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-11-17     Completed Date:  2012-03-19     Revised Date:  2013-06-30    
Medline Journal Info:
Nlm Unique ID:  7502536     Medline TA:  Br J Pharmacol     Country:  England    
Other Details:
Languages:  eng     Pagination:  1753-66     Citation Subset:  IM    
Copyright Information:
© 2011 Academic Medical Center & Rothamsted Research Ltd. British Journal of Pharmacology © 2011 The British Pharmacological Society.
Affiliation:
Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands. s.kemp@amc.uva.nl
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MeSH Terms
Descriptor/Qualifier:
ATP-Binding Cassette Transporters / genetics,  metabolism*
Animals
Disease Models, Animal
Humans
Metabolic Diseases / genetics,  metabolism
Mutation
Peroxisomes / metabolism*
Polymorphism, Single Nucleotide
Protein Transport
Tissue Distribution
Grant Support
ID/Acronym/Agency:
BB/F007299/1//Biotechnology and Biological Sciences Research Council
Comments/Corrections

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