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Updated: Jul 19, 2026
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Peroxisome Staining in Mammalian Cells Using Peroxisome-Specific Probes
Published on: December 19, 2025
The peroxisomal ABC transporter family
Ronald J A Wanders1, Wouter F Visser, Carlo W T van Roermund
1Department of Clinical Chemistry and Pediatrics, Emma Children's Hospital, Laboratory Genetic Metabolic Diseases, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
Pflugers Archiv : European Journal of Physiology
|October 14, 2006
Summary
The ABCD family of peroxisomal transporters is crucial for cellular function. Research is ongoing to define their exact roles, particularly in yeast and mammals, with implications for diseases like X-linked adrenoleukodystrophy.
Area of Science:
- Biochemistry
- Molecular Biology
- Genetics
Background:
- The ABCD family comprises peroxisomal half adenosine-triphosphate-binding cassette (ABC) transporters.
- ABCDs are found in various eukaryotic organisms, including yeast, plants, and mammals.
- Their precise functions remain largely uncharacterized.
Purpose of the Study:
- To review the current knowledge of the ABCD family of peroxisomal ABC transporters.
- To highlight the challenges in definitively establishing their functional roles.
- To discuss the implications of ABCD gene mutations in human diseases.
Main Methods:
- Literature review of existing studies on ABCD transporters.
- Analysis of data from Saccharomyces cerevisiae, Arabidopsis thaliana, and mammalian models.
- Discussion of proposed experimental approaches, such as liposome reconstitution and transport studies.
- Consideration of insights from genetically modified mouse models.
Main Results:
- Evidence suggests yeast ABCDs form a heterodimer transporting acyl-CoA esters across the peroxisomal membrane.
- Mammalian ABCD1 (adrenoleukodystrophy protein, ALDP) is linked to very-long-chain fatty acid transport.
- Mutations in ABCD1 cause X-linked adrenoleukodystrophy (X-ALD).
Conclusions:
- Definitive functional characterization of ABCDs requires successful reconstitution and transport assays.
- Understanding ABCD functions is critical for elucidating peroxisomal transport mechanisms.
- Mutant mouse models are essential tools for resolving the roles of peroxisomal half-ABC transporters.
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