The DOI matches its Crossref record; 45 of the 45 references it cites were found in a registry or an index.
Plasmalogens participate in very-long-chain fatty acid-induced pathology
Abstract
Peroxisomes are organelles responsible for multiple metabolic pathways including, the biosynthesis of plasmalogens, a class of phospholipids, and the beta-oxidation of very-long-chain fatty acids (VLCFA). Lack of peroxisomes or dysfunction in any of their normal functions is the cellular basis for human peroxisomal disorders. Here we used mouse models to understand and define the biochemical and cellular determinants that mediate the pathophysiological consequences caused by peroxisomal dysfunctions. We investigated the role and effects of cellular plasmalogens and VLCFA accumulation in liver, testis and nervous tissue using Pex7 and Abcd1 knockout (KO) mice. In addition, we also generated a Pex7:Abcd1 double KO mouse to investigate how different peroxisomal dysfunctions modulate cellular function and pathology. We found that plasmalogens function as fundamental structural phospholipids and protect cells from damage caused by VLCFA accumulation. In testis, plasmalogens protect spermatocytes from VLCFA-induced degeneration and apoptosis. In nervous tissue, we found that gliosis, inflammatory demyelination and axonopathy caused by accumulation of VLCFA are modulated by plasmalogens. Our findings demonstrate the importance of normal peroxisomal functioning and allow the understanding of the pathological causality of peroxisomal dysfunctions. Nervous tissue deficient in plasmalogens is more prone to damage, illustrating the importance of plasmalogens in peroxisomal disorders including Zellweger syndrome and X-linked adrenoleukodystrophy.
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“VLCFA accumulation might also cause demyelination by limiting peroxisomal synthesis of plasmalogens [261,262], notably plasmenyl-ethanolamine [261].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [262]
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Cahoy, J. D., Emery, B., Kaushal, A., Foo, L. C., Zamanian, J. L., Christopherson, K. S., Xing, Y., Lubischer, J. L., Krieg, P. A., Krupenko, S. A., et al. (2008) · The Journal of Neuroscience · doi:10.1523/jneurosci.4178-07.2008 · Cited by 3,129
Biochemistry of mammalian peroxisomes revisited
Wanders, R. J. A., & Waterham, H. R. (2006) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.74.082803.133329 · Cited by 995
Self-citationMoser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663
Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy
Moser, H. (1997) · Brain · doi:10.1093/brain/120.8.1485 · Cited by 478
Functions and biosynthesis of plasmalogens in health and disease
Brites, P., Waterham, H. R., & Wanders, R. J. A. (2004) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2003.12.010 · Cited by 430
Self-citationAxonal loss and neuroinflammation caused by peroxisome-deficient oligodendrocytes
Kassmann, C. M., Lappe-Siefke, C., Baes, M., Brügger, B., Mildner, A., Werner, H. B., Natt, O., Michaelis, T., Prinz, M., Frahm, J., et al. (2007) · Nature Genetics · doi:10.1038/ng2070 · Cited by 357
A mouse model for X-linked adrenoleukodystrophy
Lu, J.-F., Lawler, A. M., Watkins, P. A., Powers, J. M., Moser, A. B., Moser, H. W., & Smith, K. D. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.17.9366 · Cited by 293
A mouse model for Zellweger syndrome
Baes, M., Gressens, P., Baumgart, E., Carmeliet, P., Casteels, M., Fransen, M., Evrard, P., Fahimi, D., Declercq, P. E., Collen, D., et al. (1997) · Nature Genetics · doi:10.1038/ng0997-49 · Cited by 275
POWERS, J. M., LIU, Y., MOSER, A. B., & MOSER, H. W. (1992) · Journal of Neuropathology and Experimental Neurology · doi:10.1097/00005072-199211000-00007 · Cited by 265
Goodenowe, D. B., Cook, L. L., Liu, J., Lu, Y., Jayasinghe, D., Ahiahonu, P. W. K., Heath, D., Yamazaki, Y., Flax, J., Krenitsky, K. F., et al. (2007) · Journal of Lipid Research · doi:10.1194/jlr.p700023-jlr200 · Cited by 259
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ALTERED GLYCOLIPID METABOLISM AND ITS CELL BIOLOGICAL IMPACT IN PEROXISOMAL BIOGENESIS DISORDERS
宮崎, セ. (2014) · Institutional Repositories DataBase (IRDB) · doi:10.15083/00008996
Richert, S. (2016) · eDiss (Georg-August-Universität Göttingen) · doi:10.53846/goediss-5949
Cellular and subcellular analysis of peripheral neuropathy caused by peroxisomal dysfunction in mice
Kleinecke, S. (2016) · eDiss (Georg-August-Universität Göttingen) · doi:10.53846/goediss-5970
Ranea‐Robles, P. (2017) · Tesis Doctorals en Xarxa (Consorci de Serveis Universitaris de Catalunya)
Schuit, F. C. (2015) · Bohn Stafleu van Loghum eBooks · doi:10.1007/978-90-368-0620-6_1
Schuit, F. C. (2010) · Bohn Stafleu van Loghum eBooks · doi:10.1007/978-90-313-8225-5_1
Schuit, F. C. (2019) · Bohn Stafleu van Loghum eBooks · doi:10.1007/978-90-368-2358-6_1
Matsuda, A., Nakashima, H., Fujino, T., Okauchi, T., Hossain, M. S., Sakai, Y., Ohga, S., Nakashima, K., & Honsho, M. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.06.11.656246
Dominicci-Cotto, C., & Jenny, A. (2026) · Current topics in developmental biology/Current Topics in Developmental Biology · doi:10.1016/bs.ctdb.2026.03.005
Metadata
- Authors
- Brites, P., Mooyer, P. A. W., el Mrabet, L., Waterham, H. R., & Wanders, R. J. A.
- Affiliations
- Amsterdam UMC Location University of Amsterdam (NL)
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2008-06-20
- Volume
- 132
- Issue
- 2
- Pages
- 482-492
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2060819989
- Cited by
- 112
- References
- 45
- Citations by year
- 2026: 3 · 2025: 5 · 2024: 2 · 2023: 3 · 2022: 6 · 2021: 1 · 2020: 6 · 2019: 8 · 2018: 4 · 2017: 4 · 2016: 5 · 2015: 5 · 2014: 11 · 2013: 5 · 2012: 9
- FWCI
- 7.79
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Brain (publishedVersion)
- PubMed (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Funders
- European Commission
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
- Keywords
- plasmalogens, very long chain fatty acids, peroxisomal disorders, peroxisomes, PEX7, ABCD1, spermatocyte degeneration, apoptosis, gliosis, inflammatory demyelination, axonopathy, Zellweger syndrome, X-linked adrenoleukodystrophy, fatty acid accumulation, phospholipids
- MeSH terms
- Peroxisomal Targeting Signal 2 Receptor, Adrenoleukodystrophy, Animals, Astrocytes, Axons, Brain, Demyelinating Diseases, Fatty Acids, Male, Mice, Inbred C57BL, Plasmalogens, Blotting, Western, Microglia, Receptors, Cytoplasmic and Nuclear, Mice, Knockout, Peroxisomes, Mice, Chemokine CCL22
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