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Toxic effects of X-linked adrenoleukodystrophy-associated, very long chain fatty acids on glial cells and neurons from rat hippocampus in culture

Hein, S., Schonfeld, P., Kahlert, S., Reiser, G.·2008·Human Molecular Genetics

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Abstract

Saturated very long chain fatty acids (VLCFAs; > or =C22:0) accumulate in X-linked adrenoleukodystrophy (X-ALD, OMIM 300100), a severe hereditary neurodegenerative disease, due to peroxisomal impairment. Previous studies analysed the development of X-ALD in humans and gene knockout animal models. However, the toxic effect of VLCFA leading to severe symptoms with progressive and multifocal demyelination, adrenal insufficiency and inflammation still remains unclear. To understand the toxic effects of VLCFA in the brain, here we exposed neural cells to VLCFA and analysed the cellular consequences. We found that oligodendrocytes and astrocytes challenged with docosanoic- (C22:0), tetracosanoic- (C24:0) and hexacosanoic acids (C24:0) die within 24 h. VLCFA-induced depolarization of mitochondria in situ and increased intracellular Ca2+ level in all three brain cell types provides indications about the mechanism of toxicity of VLCFA. Interestingly, VLCFAs affect to the largest degree the myelin-producing oligodendrocytes. In isolated mitochondria, VLCFAs exert a detrimental effect by affecting the inner mitochondrial membrane and promoting the permeability transition. In conclusion, we suggest that there is a potent toxic activity of VLCFA due to dramatic cell physiological effects with mitochondrial dysfunction and Ca2+ deregulation. This provides the first evidence for mitochondrial-based cell death mechanisms in neurodegenerative disease with peroxisomal defects and subsequent VLCFA accumulation.

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Authors: Hein, S., Schonfeld, P., Kahlert, S., & Reiser, G.
Affiliations: Otto-von-Guericke-Universität Magdeburg (DE)
Corresponding author: Reiser, G.
ORCID: Kahlert, S.
Venue: Human Molecular Genetics
Publisher: Oxford University Press (OUP)
Published: 2008-03-04
Volume: 17
Issue: 12
Pages: 1750-1761
ISSN: 0964-6906 (ISSN-L)
1460-2083
DOI: 10.1093/hmg/ddn066
OpenAlex: W2167261850
Cited by: 180
References: 69
Citations by year: 2026: 10 · 2025: 5 · 2024: 17 · 2023: 6 · 2022: 11 · 2021: 13 · 2020: 15 · 2019: 4 · 2018: 11 · 2017: 7 · 2016: 11 · 2015: 10 · 2014: 8 · 2013: 15 · 2012: 9
FWCI: 5.98
Type: journal article
Language: en
Open access: closed
Locations: Human Molecular Genetics (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
Keywords: X-linked adrenoleukodystrophy, very long chain fatty acids, oligodendrocytes, astrocytes, hippocampus, mitochondrial depolarization, mitochondrial permeability transition, peroxisomal dysfunction, neurodegeneration, docosanoic acid, tetracosanoic acid, hexacosanoic acid, cell death
MeSH terms: Adrenoleukodystrophy, Animals, Astrocytes, Cells, Cultured, Fatty Acids, Hippocampus, Mitochondria, Neuroglia, Neurons, Oligodendroglia, Cell Death, Rats, Wistar, Reactive Oxygen Species, Calcium Signaling, Rats

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Toxic effects of X-linked adrenoleukodystrophy-associated, very long chain fatty acids on glial cells and neurons from rat hippocampus in culture

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