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Cited by 23Metabolism and Genetic DisordersHereditary Neurological DisordersLysosomal Storage Disorders Research

The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophy

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Coppa, Andrea, Guha, Sanjib, Fourcade, Stéphane, Parameswaran, Janani, Ruiz, Montserrat, Moser, Ann B., Schlüter, Agatha, Murphy, Michael P. +4 more·2020·Free Radical Biology and Medicine
DOI 10.1016/j.freeradbiomed.2020.01.177PMC PMC7611262
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Abstract

Adrenoleukodystrophy is a neurometabolic disorder caused by a defective peroxisomal ABCD1 transporter of very long-chain fatty acids (VLCFAs). Its pathogenesis is incompletely understood. Here we characterize a nematode model of X-ALD with loss of the pmp-4 gene, the worm orthologue of ABCD1. These mutants recapitulate the hallmarks of X-ALD: i) VLCFAs accumulation and impaired mitochondrial redox homeostasis and ii) axonal damage coupled to locomotor dysfunction. Furthermore, we identify a novel role for PMP-4 in modulating lipid droplet dynamics. Importantly, we show that the mitochondria targeted antioxidant MitoQ normalizes lipid droplets size, and prevents axonal degeneration and locomotor disability, highlighting its therapeutic potential. Moreover, PMP-4 acting solely in the hypodermis rescues axonal and locomotion abnormalities, suggesting a myelin-like role for the hypodermis in providing essential peroxisomal functions for the nematode nervous system.

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  • Loss of the pmp-4 gene, the Caenorhabditis elegans ortholog of ABCD1, induces VLCFA accumulation, axonal degeneration, and locomotor dysfunction.

    “Loss of the pmp-4 gene, the Caenorhabditis elegans ortholog of ABCD1, induces VLCFA accumulation, axonal degeneration, and locomotor dysfunction [195].”

    From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [195]

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Authors
Coppa, A., Guha, S., Fourcade, S., Parameswaran, J., Ruiz, M., Moser, A. B., Schlüter, A., Murphy, M. P., Lizcano, J. M., Miranda-Vizuete, A., Dalfó, E., & Pujol, A.
Affiliations
  • Institut d'Investigació Biomédica de Bellvitge (ES)
  • Kennedy Krieger Institute (US)
  • MRC Mitochondrial Biology Unit (GB)
  • Universitat de Vic - Universitat Central de Catalunya (ES)
  • Institució Catalana de Recerca i Estudis Avançats (ES)
Corresponding author
  • Dalfó, Esther
  • Pujol, Aurora
ORCID
Venue
Free Radical Biology and Medicine
Publisher
Elsevier BV
Published
2020-05
Volume
152
Pages
797-809
ISSN
0891-5849 (ISSN-L)
DOI
10.1016/j.freeradbiomed.2020.01.177
PMC
PMC7611262
OpenAlex
W3003186614
Cited by
23
References
20
Citations by year
2025: 3 · 2024: 3 · 2023: 1 · 2022: 7 · 2021: 8 · 2020: 1
FWCI
2.82
Type
journal article
Language
en
Open access
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • Free Radical Biology and Medicine (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
  • Apollo (University of Cambridge) (submittedVersion, cc-by-nc-nd)
  • RISalud Institutional Health Repository of Andalucía (submittedVersion, cc-by-nc-nd)
  • DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion)
  • Dipòsit Digital de la Universitat de Barcelona (Universitat de Barcelona) (submittedVersion, cc-by-nc-nd)
  • Apollo
Funders
  • CERCA Program/Generalitat de Catalunya (HYM772)
  • Government of Catalonia (2017SGR1206)
  • Spanish Ministry of Science and Competitivity (PC0009/003, PI1100968)
  • Instituto de Salud Carlos III (CPII16/00016)
  • European Social Fund
  • Center for Biomedical Research on Rare Diseases
  • Autonomous Government of Catalonia (2014FI_B2 00028)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
Keywords
adrenoleukodystrophy, ABCD1, very long chain fatty acids, peroxisomes, subcutaneous tissue, axonal degeneration, locomotor dysfunction, lipid droplets, MitoQ, mitochondrial redox homeostasis, Caenorhabditis elegans
MeSH terms
ATP Binding Cassette Transporter, Subfamily D, Member 1, Adrenoleukodystrophy, Animals, Fatty Acids, Caenorhabditis elegans, Mice, Knockout, ATP-Binding Cassette Transporters, Subcutaneous Tissue, Mice

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