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Cited by 43Metabolism and Genetic DisordersLysosomal Storage Disorders ResearchSphingolipid Metabolism and Signaling

Incidence of Abcd1 level on the induction of cell death and organelle dysfunctions triggered by very long chain fatty acids and TNF-α on oligodendrocytes and astrocytes

Baarine, Mauhamad, Ragot, Kévin, Athias, Anne, Nury, Thomas, Kattan, Zilal, Genin, Emmanuelle C., Andreoletti, Pierre, Ménétrier, Franck +3 more·2012·NeuroToxicology
DOI 10.1016/j.neuro.2011.10.007
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Abstract

X-linked adrenoleukodystrophy (X-ALD) is characterized by ABCD1 deficiency. This disease is associated with elevated concentrations of very long chain fatty acids (C24:0 and C26:0) in the plasma and tissues of patients. Under its severe form, brain demyelination and inflammation are observed. Therefore, we determined the effects of C24:0 and C26:0 on glial cells:oligodendrocytes, which synthesize myelin, and astrocytes, which participate in immune response. So, 158N murine oligodendrocytes, rat C6 glioma cells, rat primary cultures of neuronal-glial cells, and of oligodendrocytes were treated for various periods of time in the absence or presence of C24:0 and C26:0 used at plasmatic concentrations found in X-ALD patients (1-5 μM) and higher (10, 20, 40 μM). To evaluate the importance of extrinsic and intrinsic factors, the part taken by TNF-α and reduced Abcd1 level was studied. Whatever the cells considered, no effects on cell growth and/or viability were detected at 1-5 μM, more or less pronounced effects were identified at 10 μM, and an induction of cell death with increased permeability to propidium iodide and loss of transmembrane mitochondrial potential was observed at 20-40 μM. On 158N, cell death was characterized by (i) an increased superoxide anion production at the mitochondrial level; (ii) the presence of vacuoles of different sizes and shapes; a destabilization of lysosomal membrane and a cytoplasmic redistribution of lysosomes; (iii) a modulation of Abcd3/PMP70 and Acox-1 protein expression, and a decrease in catalase activity at the peroxisomal level. When TNF-α was combined with C24:0 or C26:0 and used on 158N cells, C6 cells, and on 158N cells after siRNA mediated knockdown of Abcd1, no or slight potentiation was revealed. Thus, on the different cell models used, an induction of cell death with marked cellular dysfunctions at the mitochondrial, lysosomal, and peroxisomal levels were found with C24:0 and C26:0 at 20 μM and higher. However, in our experimental conditions, plasmatic concentrations of these fatty acids were unable to induce cell death, and organelle dysfunctions on oligodendrocytes and astrocytes, and additional intrinsic and environmental factors, such as reduced Abcd1 level and/or TNF-α, were ineffective to potentiate their side effects.

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  • High VLCFA concentrations induce peroxisome loss and cell death in oligodendroglial cell lines that have lower ABCD1 expression.

    “High VLCFA concentrations induce peroxisome loss and cell death in oligodendroglial cell lines that have lower ABCD1 expression [260].”

    From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [260]

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Authors
Baarine, M., Ragot, K., Athias, A., Nury, T., Kattan, Z., Genin, E. C., Andreoletti, P., Ménétrier, F., Riedinger, J.-M., Bardou, M., & Lizard, G.
Affiliations
  • Inserm (FR)
  • Centre des Sciences du Goût et de l'Alimentation (FR)
  • Centre Georges François Leclerc (FR)
Corresponding author
Lizard, Gérard
ORCID
Venue
NeuroToxicology
Publisher
Elsevier BV
Published
2012-03
Volume
33
Issue
2
Pages
212-228
ISSN
0161-813X (ISSN-L)
DOI
10.1016/j.neuro.2011.10.007
OpenAlex
W2015185882
Cited by
43
References
84
Citations by year
2025: 2 · 2024: 1 · 2023: 1 · 2022: 3 · 2021: 3 · 2020: 3 · 2019: 4 · 2017: 3 · 2016: 2 · 2015: 10 · 2014: 4 · 2013: 3 · 2012: 4
FWCI
2.11
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • NeuroToxicology (publishedVersion)
  • PubMed (publishedVersion)
  • HAL (submittedVersion)
  • Open Repository and Bibliography (University of Liège) (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sphingolipid Metabolism and Signaling
Keywords
X-linked adrenoleukodystrophy, very long chain fatty acids, oligodendrocytes, astrocytes, TNF-α, cell death, mitochondrial dysfunction, lysosomal dysfunction, peroxisomal dysfunction, superoxide production, catalase
MeSH terms
ATP Binding Cassette Transporter, Subfamily D, Member 1, Adrenoleukodystrophy, Animals, Animals, Newborn, Astrocytes, Brain, Catalase, Cells, Cultured, Chromatography, Gas, Dose-Response Relationship, Drug, Fatty Acids, Female, Flow Cytometry, Humans, Male, Gas Chromatography-Mass Spectrometry, Mitochondria, Neurons, Oligodendroglia, RNA, Messenger, Time Factors, Tumor Necrosis Factor-alpha, Organelles, Rats, Wistar, ATP-Binding Cassette Transporters, Statistics, Nonparametric, RNA, Small Interfering, Microscopy, Electron, Transmission, Cell Size, Mice, Rats, Membrane Potential, Mitochondrial, Chemokine CCL22

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