Peroxisome deficiency but not the defect in ether lipid synthesis causes activation of the innate immune system and axonal loss in the central nervous system
Open accessAbstract
BACKGROUND: Mice with peroxisome deficiency in neural cells (Nestin-Pex5-/-) develop a neurodegenerative phenotype leading to motor and cognitive disabilities and early death. Major pathologies at the end stage of disease include severe demyelination, axonal degeneration and neuroinflammation. We now investigated the onset and progression of these pathological processes, and their potential interrelationship. In addition, the putative role of oxidative stress, the impact of plasmalogen depletion on the neurodegenerative phenotype, and the consequences of peroxisome elimination in the postnatal period were studied. METHODS: Immunohistochemistry in association with gene expression analysis was performed on Nestin-Pex5-/- mice to document demyelination, axonal damage and neuroinflammation. Also Gnpat-/- mice, with selective plasmalogen deficiency and CMV-Tx-Pex5-/- mice, with tamoxifen induced generalized loss of peroxisomes were analysed. RESULTS: Activation of the innate immune system is a very early event in the pathological process in Nestin-Pex5-/- mice which evolves in chronic neuroinflammation. The complement factor C1q, one of the earliest up regulated transcripts, was expressed on neurons and oligodendrocytes but not on microglia. Transcripts of other pro- and anti-inflammatory genes and markers of phagocytotic activity were already significantly induced before detecting pathologies with immunofluorescent staining. Demyelination, macrophage activity and axonal loss co-occurred throughout the brain. As in patients with mild peroxisome biogenesis disorders who develop regressive changes, demyelination in cerebellum and brain stem preceded major myelin loss in corpus callosum of both Nestin-Pex5-/- and CMV-Tx-Pex5-/- mice. These lesions were not accompanied by generalized oxidative stress throughout the brain. Although Gnpat-/- mice displayed dysmyelination and Purkinje cell axon damage in cerebellum, confirming previous observations, no signs of inflammation or demyelination aggravating with age were observed. CONCLUSIONS: Peroxisome inactivity triggers a fast neuroinflammatory reaction, which is not solely due to the depletion of plasmalogens. In association with myelin abnormalities this causes axon damage and loss.
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The idea that plasmalogen-deficient myelin is more prone to oxidative damage is weakened by reduced malondialdehyde and no substantial change in other oxidative stress markers in ether lipid-deficient mouse brains.
“Plasmalogen-deficient myelin might be more prone to oxidative damage [231]; however, this idea is weakened by the observations of reduced rather than elevated levels of malondialdehyde and no substantial change in other oxidative stress markers in the brains of ether lipid-deficient mice [40,73].”
From Peroxisomes in brain development and function (2016) · cites this paper as [73]
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Fu, X., Wan, P., Lü, L., Wan, Y., Liu, Z., Hong, G., Cao, S., Bi, X., Zhou, J., Qiao, R., et al. (2023) · Advanced Science · doi:10.1002/advs.202300402 · Cited by 18 · see citing works
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Semikasev, E., Ahlemeyer, B., Acker, T., Schänzer, A., & Baumgart‐Vogt, E. (2023) · Acta Neuropathologica Communications · doi:10.1186/s40478-023-01567-0 · Cited by 17 · see citing works
Beckers, L., Stroobants, S., D’Hooge, R., & Baes, M. I. (2018) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2018.00136 · Cited by 17 · see citing works
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PPAR-γImpairment Alters Peroxisome Functionality in Primary Astrocyte Cell Cultures
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Impaired plasmalogen synthesis dysregulates liver X receptor-dependent transcription in cerebellum
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Miro—Working beyond Mitochondria and Microtubules
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Central serotonergic neuron deficiency in a mouse model of Zellweger syndrome
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Dissecting Peroxisome-Mediated Signaling Pathways: A New and Exciting Research Field
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Block of a subset of sodium channels exacerbates experimental autoimmune encephalomyelitis
Stevens, M., Timmermans, S., Bottelbergs, A., Hendriks, J. J. A., Brône, B., Baes, M. I., & Tytgat, J. (2013) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2013.04.012 · Cited by 11 · see citing works
Self-citationPeroxisome Deficiency Impairs BDNF Signaling and Memory
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iBRET Screen of the ABCD1 Peroxisomal Network and Mutation-Induced Network Perturbations
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Semikasev, E., Ahlemeyer, B., Acker, T., Schänzer, A., & Baumgart‐Vogt, E. (2024) · Figshare · doi:10.6084/m9.figshare.26588829
Semikasev, E., Ahlemeyer, B., Acker, T., Schänzer, A., & Baumgart‐Vogt, E. (2024) · Figshare · doi:10.6084/m9.figshare.26588853
Semikasev, E., Ahlemeyer, B., Acker, T., Schänzer, A., & Baumgart‐Vogt, E. (2024) · Figshare · doi:10.6084/m9.figshare.26588844
Semikasev, E., Ahlemeyer, B., Acker, T., Schänzer, A., & Baumgart‐Vogt, E. (2024) · Figshare · doi:10.6084/m9.figshare.26588832
- Authors
- Bottelbergs, A., Verheijden, S., Van Veldhoven, P. P., Just, W., Devos, R., & Baes, M.
- Affiliations
- KU Leuven (BE)
- Heidelberg University (DE)
- Corresponding author
- Baes, Myriam
- Venue
- Journal of Neuroinflammation
- Published
- 2012-03-29
- Volume
- 9
- Issue
- 1
- Pages
- 61-61
- ISSN
- 1742-2094 (ISSN-L)
- PMC
- PMC3419640
- OpenAlex
- W1992716449
- Cited by
- 66
- References
- 50
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 9 · 2023: 3 · 2022: 1 · 2021: 4 · 2020: 4 · 2019: 4 · 2018: 5 · 2017: 6 · 2016: 3 · 2015: 3 · 2014: 9 · 2013: 11 · 2012: 1
- FWCI
- 3.86
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Journal of Neuroinflammation (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- heiDOK (Heidelberg University) (acceptedVersion, other-oa)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Funders
- European Commission (LSHG-CT-2004-512018, LSHG-CT-2004-512018, FP6)
- Fonds Wetenschappelijk Onderzoek (G.0760.09)
- Vlaamse regering
- Topics
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- peroxisome deficiency, PEX5, peroxisome biogenesis disorders, innate immune activation, neuroinflammation, demyelination, axonal loss, plasmalogens, GNPAT, oxidative stress, C1q, macrophages, Purkinje cell axons, cerebellum
- MeSH terms
- Peroxisome-Targeting Signal 1 Receptor, Animals, Axons, Central Nervous System, Demyelinating Diseases, Immunity, Innate, Intermediate Filament Proteins, Nerve Tissue Proteins, Plasmalogens, Receptors, Cytoplasmic and Nuclear, Mice, Knockout, Peroxisomes, Lipid Metabolism, Mice, Nestin
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