Cited by 183Metabolism and Genetic DisordersDrug Transport and Resistance MechanismsPeroxisome Proliferator-Activated Receptors

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Peroxisomal ABC transporters: Structure, function and role in disease

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Morita, Masashi, Imanaka, Tsuneo·2012·Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

DOI 10.1016/j.bbadis.2012.02.009

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Abstract

ATP-binding cassette (ABC) transporters belong to one of the largest families of membrane proteins, and are present in almost all living organisms from eubacteria to mammals. They exist on plasma membranes and intracellular compartments such as the mitochondria, peroxisomes, endoplasmic reticulum, Golgi apparatus and lysosomes, and mediate the active transport of a wide variety of substrates in a variety of different cellular processes. These include the transport of amino acids, polysaccharides, peptides, lipids and xenobiotics, including drugs and toxins. Three ABC transporters belonging to subfamily D have been identified in mammalian peroxisomes. The ABC transporters are half-size and assemble mostly as a homodimer after posttranslational transport to peroxisomal membranes. ABCD1/ALDP and ABCD2/ALDRP are suggested to be involved in the transport of very long chain acyl-CoA with differences in substrate specificity, and ABCD3/PMP70 is involved in the transport of long and branched chain acyl-CoA. ABCD1 is known to be responsible for X-linked adrenoleukodystrophy (X-ALD), an inborn error of peroxisomal β-oxidation of very long chain fatty acids. Here, we summarize recent advances and important points in our advancing understanding of how these ABC transporters target and assemble to peroxisomal membranes and perform their functions in physiological and pathological processes, including the neurodegenerative disease, X-ALD.

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Self-citation

Authors: Morita, M., & Imanaka, T.
Affiliations: University of Toyama (JP)
Corresponding author: Imanaka, Tsuneo
ORCID: Morita, Masashi
Imanaka, Tsuneo
Venue: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
Publisher: Elsevier BV
Published: 2012-09
Volume: 1822
Issue: 9
Pages: 1387-1396
ISSN: 0925-4439 (ISSN-L)
DOI: 10.1016/j.bbadis.2012.02.009
OpenAlex: W1987601583
Cited by: 183
References: 115
Citations by year: 2026: 6 · 2025: 11 · 2024: 10 · 2023: 10 · 2022: 15 · 2021: 22 · 2020: 11 · 2019: 15 · 2018: 10 · 2017: 9 · 2016: 14 · 2015: 17 · 2014: 17 · 2013: 13 · 2012: 2
FWCI: 1.86
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 366 d)
Locations: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease (publishedVersion)
PubMed (publishedVersion)
Funders: Ministry of Education, Culture, Sports, Science and Technology (20590054, 22590060, 23590072)
Ministry of Health, Labour and Welfare
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Peroxisome Proliferator-Activated Receptors
Keywords: peroxisomal ABC transporters, ABCD1, ABCD2, ABCD3, X-linked adrenoleukodystrophy, peroxisomal beta-oxidation, membrane protein targeting, neurodegenerative diseases
MeSH terms: Acyl Coenzyme A, Adrenoleukodystrophy, Animals, Biological Transport, Active, Humans, Neurons, Phylogeny, Protein Conformation, ATP-Binding Cassette Transporters, Peroxisomes, Protein Transport, Lipid Metabolism

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Peroxisomal ABC transporters: Structure, function and role in disease

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