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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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Preparation of separate astroglial and oligodendroglial cell cultures from rat cerebral tissue.
McCarthy, K. D., & de Vellis, J. (1980) · The Journal of Cell Biology · doi:10.1083/jcb.85.3.890 · Cited by 3,845
High protonic potential actuates a mechanism of production of reactive oxygen species in mitochondria
Korshunov, S. S., Skulachev, V. P., & Starkov, A. A. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)01159-9 · Cited by 1,727
Generation of reactive oxygen species by the mitochondrial electron transport chain
Liu, Y., Fiskum, G., & Schubert, D. (2002) · Journal of Neurochemistry · doi:10.1046/j.0022-3042.2002.00744.x · Cited by 1,260
Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters
Mosser, J., Douar, A.-M., Sarde, C.-O., Kioschis, P., Feil, R., Moser, H., Poustka, A.-M., Mandel, J.-L., & Aubourg, P. (1993) · Nature · doi:10.1038/361726a0 · Cited by 1,202
Peroxisomal and Mitochondrial Defects in the Cerebro-Hepato-Renal Syndrome
Goldfischer, S. L., Moore, C. L., Johnson, T., Spiro, A. J., Valsamis, M. P., Wisniewski, H. K., Ritch, R. H., Norton, W. T., Rapin, I., & Gartner, L. M. (1973) · Science · doi:10.1126/science.182.4107.62 · Cited by 791
Identification of lymphotoxin and tumor necrosis factor in multiple sclerosis lesions.
Selmaj, K. W., Raine, C. S., Cannella, B., & Brosnan, C. F. (1991) · Journal of Clinical Investigation · doi:10.1172/jci115102 · Cited by 726
Molecular mechanisms of glutamate-dependent neurodegeneration in ischemia and traumatic brain injury
Arundine, M., & Tymianski, M. (2004) · Cellular and Molecular Life Sciences (CMLS) · doi:10.1007/s00018-003-3319-x · Cited by 647
Relationship between configuration, function, and permeability in calcium-treated mitochondria.
Hunter, D. R., Haworth, R. A., & Southard, J. H. (1976) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)33220-9 · Cited by 619
Mitochondrial calcium signalling and cell death: Approaches for assessing the role of mitochondrial Ca2+ uptake in apoptosis
Hajnóczky, G., Csordás, G., Das, S., Garcı́a-Pérez, C., Saotome, M., Roy, S. S., & Yi, M. (2006) · Cell Calcium · doi:10.1016/j.ceca.2006.08.016 · Cited by 613
ΔΨ m ‐Dependent and ‐independent production of reactive oxygen species by rat brain mitochondria
Votyakova, T. V., & Reynolds, I. J. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00548.x · Cited by 601
Calcium, mitochondria and reperfusion injury: a pore way to die
Halestrap, A. P. (2006) · Biochemical Society Transactions · doi:10.1042/bst20060232 · Cited by 561
Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy
Moser, H. (1997) · Brain · doi:10.1093/brain/120.8.1485 · Cited by 478
Astrocyte apoptosis: implications for neuroprotection
Takuma, K., Baba, A., & Matsuda, T. (2004) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2004.02.001 · Cited by 443
Qualitative alteration in substrate specificity of mitochondrial monoamine oxidase in brain
Moskvitina, T. A., Kamyshanskaya, N. S., Kaverina, L. P., & Gorkin, V. Z. (1976) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1976.tb04462.x · Cited by 440
Peroxisome biogenesis disorders
Gould, S. J., & Valle, D. L. (2000) · Trends in Genetics · doi:10.1016/s0168-9525(00)02056-4 · Cited by 354
ABCD1 mutations and the X-linked adrenoleukodystrophy mutation database: Role in diagnosis and clinical correlations
Kemp, S., Pujol, A., Waterham, H. R., van Geel, B. M., Boehm, C. D., Raymond, G. V., Cutting, G. R., Wanders, R. J. A., & Moser, H. W. (2001) · Human Mutation · doi:10.1002/humu.1227 · Cited by 321
The Inflammatory Myelinopathy of Adreno-Leukodystrophy: Cells, Effector Molecules, and Pathogenetic Implications
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
[27] Primary glial cultures as a model for studying hormone action
Hamprecht, B., & Löffler, F. (1985) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/0076-6879(85)09097-8 · Cited by 250
Gene redundancy and pharmacological gene therapy: Implications for X-linked adrenoleukodystrophy
Kemp, S., Wei, H.-M., Lu, J.-F., Braiterman, L. T., McGuinness, M. C., Moser, A. B., Watkins, P. A., & Smith, K. D. (1998) · Nature Medicine · doi:10.1038/3242 · Cited by 249
Adrenoleukodystrophy: Incidence, new mutation rate, and results of extended family screening
Bezman, L., Moser, A. B., Raymond, G. V., Watkins, P. A., Smith, K. D., KASS, N. E., & Moser, H. W. (2001) · Annals of Neurology · doi:10.1002/ana.101.abs · Cited by 237
Adrenoleukodystrophy: impaired oxidation of very long chain fatty acids in white blood cells, cultured skin fibroblasts, and amniocytes
Singh, I., Moser, A. E., Moser, H. W., & Kishimoto, Y. (1984) · Pediatric Research · doi:10.1203/00006450-198403000-00016 · Cited by 234
Arachidonic acid interaction with the mitochondrial electron transport chain promotes reactive oxygen species generation
Cocco, T., Di, M., Papa, P., & Lorusso, M. P. (1999) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(99)00034-9 · Cited by 228
Mitochondrial membrane potential and hydroethidine‐monitored superoxide generation in cultured cerebellar granule cells
Budd, S. L., Castilho, R. F., & Nicholls, D. G. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)01088-0 · Cited by 222
Peroxisomal disorders I: biochemistry and genetics of peroxisome biogenesis disorders
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Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy
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Baumgart, E., Vanhorebeek, I., Grabenbauer, M., Borgers, M., Declercq, P. E., Fahimi, H. D., & Baes, M. (2001) · The American Journal of Pathology · doi:10.1016/s0002-9440(10)62534-5 · Cited by 210
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Ho, J. K., Moser, H., Kishimoto, Y., & Hamilton, J. A. (1995) · Journal of Clinical Investigation · doi:10.1172/jci118182 · Cited by 199
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Smith, K. D., Kemp, S., Braiterman, L. T., Lu, J.-F., Wei, H.-M., Geraghty, M., Stetten, G., Bergin, J. S., Pevsner, J., & Watkins, P. A. (1999) · Neurochemical Research · doi:10.1023/a:1022535930009 · Cited by 190
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Verhoeven‐Duif, N. M., Roe, D. S., Kok, R. M., Wanders, R. J. A., Jakobs, C., & Roe, C. R. (1998) · Journal of Lipid Research · doi:10.1016/s0022-2275(20)34204-8 · Cited by 131
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Wanders, R. J. A., & Tager, J. M. (1998) · Molecular Aspects of Medicine · doi:10.1016/s0098-2997(98)00003-x · Cited by 122
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Dubois-Dalcq, M., Feigenbaum, V., & Aubourg, P. (1999) · Trends in Neurosciences · doi:10.1016/s0166-2236(98)01319-8 · Cited by 121
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Theda, C., Moser, A. B., Powers, J. M., & Moser, H. W. (1992) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(92)90028-j · Cited by 99
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van Geel, B. M., Assies, J., Weverling, G. J., & Barth, P. G. (1994) · Neurology · doi:10.1212/wnl.44.12.2343 · Cited by 90
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Bernardi, P., Penzo, D., & Wojtczak, L. (2002) · Vitamins and hormones · doi:10.1016/s0083-6729(02)65061-7 · Cited by 85
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Wanders, R. J. A., van Roermund, C. W. T., Lageweg, W., Jakobs, B. S., Schutgens, R. B. H., Nijenhuis, A. A., & Tager, J. M. (1992) · Journal of Inherited Metabolic Disease · doi:10.1007/bf01799620 · Cited by 51
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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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