Cited by 783Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsHereditary Neurological Disorders

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Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

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Haidet-Phillips, Amanda M, Hester, Mark E, Miranda, Carlos J, Meyer, Kathrin, Braun, Lyndsey, Frakes, Ashley, Song, SungWon, Likhite, Shibi, Murtha, Matthew J, Foust, Kevin D, Rao, Meghan, Eagle, Amy, Kammesheidt, Anja, Christensen, Ashley, Mendell, Jerry R, Burghes, Arthur H M, Kaspar, Brian K·2011·Nature Biotechnology

DOI 10.1038/nbt.1957PMC PMC3170425

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Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, with astrocytes implicated as contributing substantially to motor neuron death in familial (F)ALS. However, the proposed role of astrocytes in the pathology of ALS derives in part from rodent models of FALS based upon dominant mutations within the superoxide dismutase 1 (SOD1) gene, which account for <2% of all ALS cases. Their role in sporadic (S)ALS, which affects >90% of ALS patients, remains to be established. Using astrocytes generated from postmortem tissue from both FALS and SALS patients, we show that astrocytes derived from both patient groups are similarly toxic to motor neurons. We also demonstrate that SOD1 is a viable target for SALS, as its knockdown significantly attenuates astrocyte-mediated toxicity toward motor neurons. Our data highlight astrocytes as a non-cell autonomous component in SALS and provide an in vitro model system to investigate common disease mechanisms and evaluate potential therapies for SALS and FALS.

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Authors: Haidet-Phillips, A. M., Hester, M. E., Miranda, C. J., Meyer, K., Braun, L., Frakes, A., Song, S., Likhite, S., Murtha, M. J., Foust, K. D., Rao, M., Eagle, A., Kammesheidt, A., Christensen, A., Mendell, J. R., Burghes, A. H. M., & Kaspar, B. K.
Affiliations: Nationwide Children's Hospital (US)
Ambry Genetics (United States) (US)
The Ohio State University (US)
Corresponding author: Kaspar, Brian K
ORCID: Hester, Mark E
Miranda, Carlos J
Meyer, Kathrin
Likhite, Shibi
Foust, Kevin D
Mendell, Jerry R
Burghes, Arthur H M
Kaspar, Brian K
Venue: Nature Biotechnology
Publisher: Springer Science and Business Media LLC
Published: 2011-08-10
Volume: 29
Issue: 9
Pages: 824-828
ISSN: 0733-222X (ISSN-L)
1087-0156
1546-1696
DOI: 10.1038/nbt.1957
PMC: PMC3170425
OpenAlex: W2059355164
Cited by: 782
References: 30
Citations by year: 2026: 21 · 2025: 29 · 2024: 43 · 2023: 38 · 2022: 54 · 2021: 46 · 2020: 68 · 2019: 59 · 2018: 55 · 2017: 58 · 2016: 66 · 2015: 59 · 2014: 70 · 2013: 59 · 2012: 50
FWCI: 37.69
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: www.springer.com/tdm (tdm)
Locations: Nature Biotechnology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: National Institute of Neurological Disorders and Stroke (RC2NS069476, R01 NS644912-1A1, RC2 NS69476-01, R01NS064492, T32NS077984, F31NS058224)
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords: amyotrophic lateral sclerosis, astrocytes, motor neuron toxicity, sporadic ALS, familial ALS, SOD1, disease mechanisms
MeSH terms: Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Cell Differentiation, Cell Line, Disease Models, Animal, Gene Expression Regulation, Humans, Immunohistochemistry, Motor Neurons, Mutation, Superoxide Dismutase, Signal Transduction, Biomarkers, Sequence Analysis, DNA, Coculture Techniques, Mice, Embryonic Stem Cells, Mesenchymal Stem Cells

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Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

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