| Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance. | |
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MedLine Citation:
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PMID: 21488864 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Stephan Kemp; Frederica L Theodoulou; Ronald J A Wanders |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: British journal of pharmacology Volume: 164 ISSN: 1476-5381 ISO Abbreviation: Br. J. Pharmacol. Publication Date: 2011 Dec |
Date Detail:
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Created Date: 2011-11-17 Completed Date: 2012-03-19 Revised Date: 2013-05-24 |
Medline Journal Info:
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Nlm Unique ID: 7502536 Medline TA: Br J Pharmacol Country: England |
Other Details:
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Languages: eng Pagination: 1753-66 Citation Subset: IM |
Copyright Information:
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© 2011 Academic Medical Center & Rothamsted Research Ltd. British Journal of Pharmacology © 2011 The British Pharmacological Society. |
Affiliation:
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Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands. s.kemp@amc.uva.nl |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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ATP-Binding Cassette Transporters
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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:
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BB/F007299/1//Biotechnology and Biological Sciences Research Council |
| Comments/Corrections | |
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